Barnhills Dermatopathology 4Th Edition Raymond L Barnhill Ed Full Chapter
Barnhills Dermatopathology 4Th Edition Raymond L Barnhill Ed Full Chapter
Barnhills Dermatopathology 4Th Edition Raymond L Barnhill Ed Full Chapter
Editor In Chief
Raymond L. Barnhill, MD
Professor of Pathology
lnstitut Curie
University of Paris Descartes
Paris, France
Auocfatw Edlton
A. Neil Crowson, MD
Clinical Professor of Dermatology, Pathology and Surgery
Director of Dermatopathology
University of Oklahoma
President
Pathology Laboratory Associates
Tulsa, Oklahoma
Cynthia M. Magro, MD
Distinguished Professor of Pathology and Laboratory Medicine
Weill Cornell Medicine
New York., New York
Michael W. Piepkorn, MD
Clinical Professor of Dermatology
Department of Medicine, University of Washington School of Medicine
Seattle, Washington
Garrett T. Desman, MD
Associate Program Director, Pathology Residency Program
Attending Pathologist, Dermatopathology Division
Assistant Professor of Pathology, Molecular and Cell-Based Medicine
Assistant Professor of Dermatology
Icahn School of Medicine at Mount Sinai
NewYork, NewYork
New York Chicago San Francisco Athens London Madrid Mexico City
Milan New Delhi Singapore Sydney Toronto
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To Claire
Contents
•Additional content for Chapters 12 and 38 along with the full text for Appendix 4 can be found at: mhprofessional.com/
barnhillsdermatopathology4e.
Contributors
Michael W. Piepkorn, MD Thomas J. Walsh, MD, PhD (Hon), FIDSA, FAAM, FECMM
Clinical Professor of Dermatology Founding Director, Transplantation-Oncology Infectious
Department of Medicine, University of Washington Diseases Program
School of Medicine Chief, Infectious Diseases Translational Research Laboratory
Seattle, Washington Professor of Medicine, Pediatrics, and Microbiology and
Chapter 14. Alterations of the Stratum Corneum and Immunology
Epidermis Weill Cornell Medicine of Cornell University,
Chapter 27. Tumors of Melanocytes New York Presbyterian Hospital, and Hospital for Special
Surgery
Melissa Pulitzer, MD Henry Schueler Foundation Scholar
Associate Attending Pathologist Investigator of Emerging Infectious Diseases of the Save Our
Memorial Sloan Kettering Cancer Center Sick Kids Foundation
Assistant Professor Adjunct Professor of Pathology, Johns Hopkins University
Weill Medical College of Cornell University School of Medicine
New York, New York Adjunct Professor of Medicine, University of Maryland School
Chapter 34. Lymphoid, Leukemic, and Other Cellular of Medicine
Infiltrates Visiting Professor, National and Kapodistrian University of
Athens Medical Schoo~ Athens, Greece
Ronald P. Rapini, MD Chapter 21. Fungal Infections
Professor and Chernosky Distinguished Chair
Department of Dermatology Michel Wassef, MD
Professor of Pathology Associate Professor of Pathology
University of Texas McGovern Medical School at Houston Hopital Lariboisiere
MD Anderson Cancer Center Universite Paris Diderot-Assistance Publique H6pitaux
Houston, Texas de Paris
Chapter 19. Bacterial Infections Paris, France
Chapter 31. Vascular Tumors and Vascular Malformations
Franco Rongioletti, MD
Full Professor and Chairman Kelly L. West, MD, PhD
Unit of Dermatology Director of Research and Education
University of Cagliari Ball Derrnpath, PA
Cagliari, Italy Greensboro, North Carolina
Chapter 16. Deposition Disorders Chapter 38. Disorders of the Genital Mucosa
xii CONTRIBUTORS
As senior editor of this new Textbook of Dermatopathology, both established leaders in the field as well as the cream of our
Dr. Raymond Barnhill brings to the task years of experience as a younger generation of dermatopathologists. He himself has
dermatopathologist, clinical dermatologist, clinical investigator, coauthored one-third of the chapters of the book. and I know
and author. This has given him an unparalleled understanding firsthand that he has spent a great deal of effort meticulously
of inflammatory and neoplastic diseases of the skin and the editing all chapters for consistency, style, and accuracy. His
ability to teach others. As director of dermatopathology at the associate editor, Dr. Neil Crowson, has been instrumental in
Brigham and Women's Hospital and the Children's Hospital providing the high-quality micrographs for many of the entities
in Boston, he has had contact with both pediatric and adult in the book, and two young. energetic assistant editors, Ors.
dermatologic conditions, and as a member of the Combined Klaus Busam and Scott Granter, contributed heavily to the
Harvard DermatopathologyTraining Program, he has developed book.
the knowledge and skills to interact with pathologists, derma- I look forward to the publication of this text and wish it the
tologists, trainees, and students. The success of his monograph success it truly deserves.
on melanocytic lesions of the skin speaks to the effectiveness of
his writing and teaching approaches. Ramzi. S. C.Otran, MD, 1998
For this book, Dr. Barnhill has assembled a stellar roster of
authors from various disciplines and has appropriately included
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Preface to the Fourth Edition
The editor and publisher are extremely enthusiastic to offer a existing black and white and many color images, increasing
fourth edition of this book to physicians and others in the fields the overall number of photographs, and finally including
of dermatopathology, dermatology, and allied specialties. It is new clinical images. All chapters and the appendices have
the express wish of the editors, contributors, and the publisher been superbly revised, and much new information has been
that the information compiled in this work greatly aids phy- included with respect to various inflammatory conditions,
sicians and other health care workers in facilitating the best infections, melanocytic. lymphoid, vascular, and other neo-
patient care possible. The editor is deeply appreciative of the plastic conditions. Two entirely new superlative chapters
prodigious work of the associate editors Neil Crowson, Cynthia concerning disorders of the genital mucosa and tumors of
Magro, Michael Piepkom, Heinz Kutzner, and Garrett Des.man the conjunctiva have been included. Newly described entities
and the many eminent contributors in producing an accessible have been added to the book where appropriate, and many
and scholarly book. new color photomicrographs have been incorporated into the
The fourth edition has been revised with the same goals as fourth edition.
the previous editions-that is, to provide "descriptive histopa- As before, I aclcnowledge the tremendous efforts of the many
thology and differential diagnosis, critical analysis, balanced people involved in this fourth edition, without which it would
perspectives on what is known and what is not, clarity of writ- not have been possible. I am especially indebted to all the con-
ing, the use of tables to summarize the key features of major tributors and to the staff at McGraw-Hill who have made the
entities, and color photomicrographs" and also to maintain a fourth edition a reality. I sincerely hope that the information
uniform style. contained in this book may contribute to improved and more
In the course of revising the book. a significant effort has enlightened patient care.
been made to improve the overall quality of photographs in
the book. The latter has been accomplished by replacing the Ra')'DlOnd L Barnhill, MD
Preface to the First Edition
The question might be posed, "Why another textbook of der- and describes the use of transverse sections in the diagnosis of
matopathology?" since a number of books are currently avail- alopecia. Dr. Crowson has written a comprehensive chapter on
able and would seem to do justice to the subject. Quite simply, drug eruptions and included lists of medications implicated in
I have perceived the need for another book. Following on the these eruptions. Critical chapters on controversial and difficult
success of a monograph on melanocytic lesions of the skin, topics such as vasculitis, panniculitis, disorders of pigmenta-
The Pathology of Melanocytic Nevi and Malignant Mel.anoma, tion, and melanocytic lesions will provide greater insight and
I believe that there is indeed a need for a general text on dermato- aid to the pathologist dealing with these conditions. Finally,
pathology emphasizing the same format as the aforementioned there are more detailed chapters on disorders of the nails and
monograph: descriptive histopathology and differential diag- the oral mucosa than are currently available in most other texts.
nosis, critical analysis, balanced perspectives on what is known Another modification has been the inclusion of a scholarly
and what is not, clarity of writing. the use of tables to summarize section on normal skin histology, laboratory methods, immu-
the key features of major entities, and color photomicrographs. nohistochemistry, and the molecular biology ofcutaneous lym-
At the same time it must be acknowledged that the scope of phoid infiltrates in an appendix rather than in the text itself.
such a book goes well beyond that of a monograph on melano- It will become evident to the reader that there is occasion-
cytic lesions. As a result, I have engaged a scholarly group of ally some overlap or duplication of some conditions among the
individuals to help in writing such a book in a timely fashion. various chapters, since no method of classification is entirely
Nonetheless, one of my major goals has been to maintain a uni- consistent. My intention has been to allow some duplication
form style in keeping with the philosophy of the book. since this provides different perspectives on a disease process.
In recent years I have been impressed with the need to pro- Finally and most importantly, I would like to acknowledge
vide some orientation for beginning the process oflearning der- the tremendous efforts of many friends and colleagues with-
matopathology. Thus, the first chapter of this book is devoted out whose advice, encouragement, and help this book would
to the approach to diagnosis at the microscope. Daniel Jones, not have been possible. First of all, I would like to thank
MD, PhD, also discusses succinctly the scientific basis of pat- Neil Crowson for his enormous contn"butions in photography
tern recognition as a prologue to algorithms and the description and writing and for bis unflagging encouragement and support
of the major patterns of inflammation of the skin. Christopher throughout the project. I am also indebted to my former fellows
French, MD, has, in addition, designed schematic color figures Klaus Busam and Scott Granter for their commitment and hard
that enhance the recognition of patterns of inflammation of work on the book, and I am most appreciative of my secretaries
the skin. Another major feature is that associate editor Neil Robin McCarthy (who has since departed for an undoubtedly
Crowson. MD, has taken high-quality photomicrographs for easier job!) and Maria Palaima. and the staff at McGraw-Hill
most of the entities in the book. This is another characteristic for their dedication and efforts in bringing the book to closure.
that provides a uniform style to the book. I am deeply grateful Lastly, I am most grateful to all the contributors who have sac-
to Dr. Crowson for this enormous undertaking. rificed so much of their time and energy to make the book not
Although all major entities have been covered in an eru- only possible but a learned work that will have an impact on
dite fashion in the book, a number of unique features must the field.
be mentioned. The chapter on disorders of the skin append-
ages provides new quantitative information on the alopecias Raymond L. Barnhill, MD
Table of Acronyms and
Abbreviations
p PSRHC: primary signet ring cell/histiocytoid SFT: soft tissue tumor/solitary fibrous tumor
PAM: primary acquired melanosis carcinoma of the skin SH: sebaceous hyperplasia
PAM/C-MIN: conjunc:tival primary acquired PTL: primary cutaneous peripheral T-cell SIN: squamous intraepithelial neoplasia
melanosis/melanoc:ytic intraepithelial lymphoma SJS: Stevens-Johnson syndrome
neoplasia PTT: proliferating tricholemmal tumor/cyst SL: solar lentigo
PAMS: paraneoplastic autoimmune PUPPP: pruritic urticarial papules and SLE: systemic lupus erythematosus
multiorgan syndrome plaques of pregnancy SM: systemic mastocytosis
PAN: polyarteritis nodosa PUVA: psoralen and ultraviolet A SMA: smooth muscle actin
PAPA: pyogenic arthritis, pyoderma photochemotherapy SNP: single nucleotide polymorphism
gangrenoswn and ac:ne syndrome PVPN: pseudoverrucous papules and nodules SOXlO: SRY-related HMG-box 10
PAPASH: pyogenic arthritis, pyoderma of infancy SP: squamous papilloma
gangrenoswn and ac:ne syndrome with PWSO: proximal white subungual SPP: small-plaque parapsoriasis
hidradenitis suppurativa onychomycosis SPTCL: subcutaneous panniculitis-like T-cell
PAS: periodic acid-Schiff lymphoma
pc:ALCL: primary cutaneous anaplastic large Q SR: solitary reticulohistiocytoma
cell lymphoma qPCR: quantitative polymerase chain reaction SS: Sezary syndrome
PCH: penile cutaneous horn SSCP: single-strand DNA conformational
PCM: plasma cell mucositis R polymorphism
PCR: polymerase chain reaction RA: reactive arthritis SSM: superficial spreading melanoma
PEC: proliferating epithelial cyst RAE: reactive angioendotheliomatosis SSSS: staphylococcal scalded-skin syndrome
PEH: pseudoepitheliomatous hyperplasia RAPK: reticulate acropigmentation of SVV: small-vessel vasculitis
PEM: pigmented epithelioid Kitamura
melanocytoma RASD: reticulated acanthoma with sebaceous T
PEP: polymorphic eruption of pregnancy differentiation TA: tufted angioma
PF: perforating folliculitis REM: reticular erythematous mucinosis TB: tuberculosis
PFAPA: periodic fever, adenitis, pharyngitis, RH: retiform hemangioendothelioma TCR: T-cell receptor
aphthae RICH: rapidly involuting congenital TdT: terminal deoxynucleotidyl transferase
PG: pyogenic granuloma hemangioma TEN: toxic epidermal nec:rolysis
PH: papillary hemangioma RLH: reactive lymphoid hyperplasia TFI: tumor of the follicular infundibulwn
PHE: pseudomyogenic RMS: rhabdomyosarcoma TGGE: temperature gradient gel
hemangioendothelioma RMSF: Rocky Mountain Spotted Fever electrophoresis
PILA: papillary intralymphatic RS: Reed-Sternberg TGU: telogen germinal unit
angioendothelioma RT-PCR: real-time polymerase chain THH: targetoid hemosiderotic hemangioma
PKMB: pseudoepitheliomatous, keratotic, and reaction/reverse transcription polymerase TMEP: telangiectasia macularis eruptiva
micaceous balanitis chain reaction perstans
PLAID: PLC gamma 2-associated antibody RXLI: X-linked recessive ichthyosis T-PLL: T-cell prolymphocytic leukemia
deficiency and immune dysregulation TRAPS: TNF receptor-associated periodic
PLC: pityriasis lichenoides chronica s syndrome
PLEVA: pityriasis lichenoides et varioliformis SA: sebaceous adenoma TSS: toxic shock syndrome
acuta SADDAN: severe achondroplasia with TTM: trichotillomania
PLEXSCN: plexiform spindle cell nevus developmental delay and acanthosis TUG: traumatic ulcerative granuloma
PM: pediatric melanoma/pilomatricoma nigricans
PNECS: primary neuroendocrine carcinoma SALP: stasis-associated lipomembranous u
of the skin panniculitis uPeIN: usual penile intraepithelial neoplasia
PNH: progressive nodular histiocytoma SAP: serum amyloid P uVeIN: usual vulvar intraepithelial neoplasia
PNP: paraneoplastic: pemphigus SAVI: sting associated vasculopathy with
PP: palmar psoriasis onset in infancy v
PPD: pigmented purpuric dermatosis SC: sebaceous carcinoma VH: verrucous hemangioma
PPP: pearly penile papule SCC: squamous cell carcinoma VP: vestibular papillomatosis
PR: pagetoid reticulosis SCH: spindle cell hemangioma VSCC: vulvar squamous cell carcinoma
PRP: pityriasis rubra pilaris SCLE: subacute cutaneous lupus V'ZV: varicella-zoster virus
PSA: pilar sheath acanthoma erythematosus
PSCC: penile squamous cell carcinoma SCL/PL: spindle cell/pleomorphic lipoma w
PSCN: pigmented spindle cell nevus SCSCC: spindle cell squamous cell carcinoma WCD: Weber-Christian disease
PSL: pseudolymphoma SDM: solitary dermal melanoma WDL: well-differentiated liposarcoma
PSO: proximal subungual onychomycosis SEDC: squamoid eccrine ductal carcinoma WSO: white superficial onychomycosis
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PART 1
Inflammatory Reactions
in the Skin
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Introduction
to Microscopic
Interpretation CHAPTER 1
Raymond L. Barnhill • Daniel M. Jones • Almut Boer-Auer
usually are obtained for diagnosis of keratoses or basal or a "square" punch biopsy versus the typical inverted cone
squamous cell carcinoma. In general, punch biopsies are configuration, thickening, or atrophy of the dermis or
submitted for diagnosis of either neoplastic or inflamma- deposition of material such as calcium. A primary prolif-
tory conditions. In some instances they are used to "excise" erative or neoplastic condition also should be obvious in
a proliferation or tumor, and thus margins may need to be most instances at scanning magnification.
assessed. By and large, skin ellipses (excisions) are submit- After the completion of this exercise, the pathologist is often
ted for suspected tumors but also on occasion for inflam- able to establish the basic nature and localization of the disease
matory processes such as vasculitis or panniculitis. process and possibly to develop a preliminary differential diag-
2. Next, the pathologist should inspect the specimen with the nosis if he or she has not already arrived at a specific diagnosis.
idea of determining in general terms from what anatomic In many instances, this may not be possible at scanning mag-
site the tissue was taken. In general, based on character- nification because the changes are too subtle for diagnosis at
istics such as prominence of sebaceous follicles, relative this or perhaps any magnification or there has been a sampling
paucity of hair follicles, thickness of the reticular dermis, error. At this point, the pathologist must go to greater magnifi-
and thickness of the stratum corneum, one can recognize cation in order to confirm an impression or to gain more infor-
the following general regions of the integument: (a) head mation that is only possible at increased magnification.
and neck, (b) trunk and proximal extremities, and (c) acral
(including frictional) surfaces. It is evident that many dis- Examination at intermediate magnification
eases have characteristic site distributions, and knowledge It cannot be overemphasized that, in general, most information
of the particular localization of the lesion or eruption is about a pathologic process is obtained at scanning magnifi-
useful in formulating one's differential diagnosis. cation. The tendency to go to higher magnification too soon
3. The entire specimen (ie, epidermis, dermis, or subcutis) should be resisted because one often will overlook a crucial fea-
should be scanned for the principal site of involvement ture, and thus, in effect, one "cannot see the forest for the trees."
by a disease process, if any, and the nature of the pro- The reasons for closer inspection of the specimen (with 1Ox and
cess, whether inflammatory, proliferative, inflammatory 20x objectives) are to confirm particular features of pathologic
and proliferative, or noninflammatory. Although in most processes, for example, parakeratosis, spongiosis, fibrinoid
instances the site of involvement is obvious, it is impor- necrosis, or mucin deposition, and for identification of spe-
tant that the specimen is examined systematically when the cific cell types, such as lymphocytes or granulocytes. However,
process is not so obvious. In general, the specimen should in some instances, greater magnification may be needed in
be scrutinized in a sequential fashion, for example, begin- order to identify a morphologic feature not recognizable at low
ning with the stratum corneum and then proceeding to the magnification, such as hyphal elements in the cornified layer,
epidennis, dermis, subcutis, and fascia. At scanning mag- epidermal basal layer vacuolopathy, amyloid deposits in the
nification, one should be able to appreciate many aspects papillary dermis, or mucinosis in the reticular dermis.
of the disease process without going into greater magnifi-
cation. If an inflammatory process is present, one should Examination at high magnification
attempt to recognize the nature of epidermal involve- As mentioned for intermediate magnification, use of the high-
ment, for example, spongiosis, interface vacuolopathy, power objective also should be reserved for specific indications.
psoriasiform epidermal hyperplasia, or vesicle, blister, or Such examination is necessary in order to study the cytologic
pustule formation; the pattern of the inflammatory infil- details of cells, such as the nuclear contours oflymphocytes or
trate, whether bandlike (cell-poor or cell-rich/lichenoid), nuclear atypia in general, to confirm the nature of infectious
perivascular, interstitial, periadnexal, nodular, or diffuse organisms, and to confirm other findings.
(pandermal); the depth of the infiltrate, for example, super-
ficial only or superficial and deep; possibly the presence of Integration of all information
vascular damage; the cellular composition of an infiltrate- During the preceding exercise of examining the microslide, the
that is, whether it is comprised of mononuclear cells, sug- histopathologist should take the perspective that he or she is
gesting small lymphocytes or larger cells; and alterations of objectively gathering information that can be integrated with
the dermis, for example, by fibrosis or sclerosis resulting in other (clinical and laboratory) information to arrive at some
Chapter 1 INTRODUCTION TO MICROSCOPIC INTERPRETATION 5
conclusion about the disease process and not necessarily to biopsy specimen is inadequate, or sampling error has occurred.
arrive at a single diagnosis. One should, at all times, try to avoid In this case, inclusion of pertinent negative findings may be
reaching a conclusion too quickly and failing to observe other helpful in guiding the next step in the workup.7
pertinent findings in the specimen. The pathologist always
should try to think expansively of every potential pathologic PATIERN RECOGNITION IN
process that might explain the histologic findings. One should DERMATOPATHOLOGY
continuously weigh the various points that argue for or against
a particular pathologic condition. Accurate perception of the findings on a microslide is obviously
After completing the preceding examination and reaching the initial step in accurate diagnosis. Many, perhaps most, seri-
some tentative impression (or lack of conclusion) about the ous errors in diagnosis are related to a failure to attend to or
specimen without knowledge of clinical parameters, it is then notice critical histologic findings rather than a misinterpreta-
necessary to consider the clinical context of the specimen. Even tion of these features. A trivial but common reason for misdiag-
if the histopathologic diagnosis appears straightforward, such nosis is simply a failure to examine a relevant tissue fragment or
as, for example, basal cell carcinoma, the pathologist always level Poor tissue preservation and histologic detail or substan-
should have certain clinical information before finalizing the dard tissue sectioning also can contribute greatly to problems in
case: age, gender, anatomic site, an accurate clinical descrip- accurate perception. The visual fatigue and information over-
tion of the lesion(s) or eruption, pertinent brief clinical history load that occur after examining many cases also contributes to
if relevant. and clinical differential diagnosis. If at all possible, perceptual mistakes.
clinical photographs should be provided by clinicians. With- As outlined in Fig. 1-1, the initial decision one must make
out such information, the pathologist is much more prone to is whether a process is predominantly inflammatory, predom-
blatant errors, such as rnislabeled specimens or misdiagnosis." inantly proliferative/neoplastic, both inflammatory and pro-
For many conditions, particularly inflammatory processes liferative, or noninflamm.atory/nonproliferative. This is not
and melanocytic lesions, detailed clinical information concern- always possible, but, in general, the vast majority of pathologic
ing the onset. evolution, distribution, and specific character of processes can be categorized into one of these groups. Thus one
the skin lesions is essential in order to arrive at a diagnosis or is able to proceed along one of the decision trees.
to formulate a differential diagnosis. Many inflammatory reac-
tion patterns are not specific and may be secondary to several Features of benign venus malignant tumors
processes. Thus an accurate clinical history is needed to estab- Although it may appear intuitive and instantaneous, recogni-
lish the most likely condition or group of conditions that might tion ofcutaneous tumors is a highly complex perceptual process
explain the histologic findings. The pathologist is encouraged that involves more than simple "wallpaper matching." Particu-
to communicate directly with the clinician in order to achieve larly in the diagnosis of cutaneous tum.ors, the low-power pat-
optimal clinicopathologic correlation. tern (tumor silhcnutte) often can be at least as important if not
Finally the histopathologist must recognize the limitations definitive for diagnosis as high-power cytologic findings.,
of histopathological interpretation: in some instances specific Certain tumor growth patterns appear to be intrinsically
diagnosis is not possible including because histological find- more readily recognizable and often are the first to be mas-
ings are nonspecific and diagnosis rests in the clinical domain, tered by the student of dermatopathology. Easy recognition
current knowledge about the disease process is inadequate, the of some benign skin tum.ors (Fig. 1-2, eg, seborrheic keratosis,
FIGURE 1-2 DJstlngulshlng benfgn from malignant neoplasms by low-power microscopic features. Well circumscribed
epithelial proliferation with characteristic acan1hosis, hyperkeratosis, and keratin pseudocyst formation in seborrheic keratosis
(left). Asymmetric epithelial proliferation with infiltrating growth pattem and formation of parakeratotic cysts in cutaneous
squamous cell carcinoma (right).
6 OERMATOPATHOLOGY
FIGURE 1..3 Examples of poorly-circumscribed dennal prollferat.tons that are more dlfftcult to recognize. A sclerosing blue
nevus {left) and a desmoplastic melanoma (right). Prominent and irregular involvement of the subcutaneous tissue and patchy
lyrnphocytic infiltrates seen at scanning magnification provide support for desmoplastic melanoma {right).
ke.ramacanthoma. or cylindroma) in contrast to others (Pig. 1-3, is descriptive (ie, what kinds of epidermal alterations and
eg. a sclerosing melanocytic nevus or desmoplastic me1anoma)1 inflammatory cells are present and where they are located). In
is due in part to the visual impact of their particular growth the absence ofadequate clinical information, a range ofpossible
patterns evident at low magnmcation. diagnoses usually is provided. This is a different perceptual task
Similarly, the presence of ancillary features such as inflam- where the goal is not simply to match a precise stored visual
matory infiltrate can be an instantly recognizable clue to search image but to compare the features of a lesion with the clinical
for evidence of twnor invasion. As a pathologist gains experi- entity to which it is (statistically) most similar.
ence with twnors in dermatopathology, the distinction of the Inflammatory lesions are grouped initially into general cate-
range of benign versus malignant growth patterns becomes gories (see Pigs. 1-4 to 1-8), and then specific features are sought
easier. to narrow (or prioritize) the diagnoses. For instance, once the
pattern of vacuolar interface dermatitis has been identified,
Features of inflammatory and reactive lesions visual search for viral inclusions or apoptotic bodies could sup-
Although it also involves pattern recognition, the diagnosis port the diagnoses ofa viral exanthem or erythema multiforme.
of inflammatory lesions presents a much different challenge. Combining the available information on the gross appear-
Instead of rendering a definitive diagnosis, the usual approach ance and the clinical differential diagnosis with the histologic
i !
Intraepidermal Subepidermal Vacuolar Licbenoid
Chap. 7 Chap.8 type type
!
Iunctional Dermolytic
.. ! ! ! !
Subcomeal
lnlnalratum Suprabuilar, Conventional Bosinophilic Neutrophilic Follicular Miliarial
11Ub91ratu.m
spinosum intrabasilar spongiosis spongiosia spongiosis spongiosis spongiosis
granulosum
FIGURE 1-5 Inflammatory conditions of the dermis and/or subcutis without epidermal alterations and with and without
vascular injury.
Granulomas/granulomatous inflammation
Chap.6
~
Hyperkeratosis
Alteration of
with parakeratosis Hyperorthokeratosis
basal layer melanin
focal or diffuse
l l
Normal granular
Hypogranulosis layer or
hypergranulosis
l l
Hypopigmentation, Hyperpigmentation,
focal or diffuse focal or diffuse
Chap.15 Chap. 15
FIGURE 1-7 Noninflammatory disorders with alterations of stratum corneum and epidermis.
I OERMATOPATHOLOGY
I
! !
Deposition
!
Hypertrophy
!
Pigmentary
Atrophy of material fibrosis alterations
Chap.16 Chap.17 Chap.15
diagnosis is the vitally important penultimate stage prior to 3. Etiological relationships between disease entities. For
rendering a final diagnosis. The close linkage in training and example, cutaneous T-cell lymphomas may evolve from a
expertize between dermatologist and dermatopathologist often preceding atopic dermatitis. Therefore, there can be over-
makes this process easier because the histopathologist becomes lap in the histologic features requiring careful attention to
more familiar with the clinical appearance of the lesions in the lymphocyte atypia at high magnification.5
differential diagnosis. This synergistic effect is most obvious in Full maturation of expertize in dermatopathology thus
the rare cases where the biopsy specimen is being diagnosed by requires not only a "library" of stored visual images of inflam-
the person who has actually examined the patient. However, in matory skin lesions that correlate with. clinical syndromes but
all cases, the differential diagnosis provided by the submitting also knowledge of the time-course and spectrum. of changes
dermatologist may force a reexamination of the mic:roscopic seen for each particular entity. As discussed below, the stepwise
features, and as a result, this book provides detailed descrip- approach to learning to diagnose inflammatory lesions in skin
tions of both the histological features and clinical appearance emphasizes the importance of mastering the differential diag-
of each entity. nosis for each inflammatory pattern.
There are also added complexities to diagnosis of inflam-
matory lesions in the skin that are related to the nature of the Major inflammatory reaction pattems in the skin
immune responses producing these lesions. These include: Since the pathogenesis of most inflammatory dermatitides is
1. The temporal phase of a lesion. Many dermatitis reactions unknown, one must, of necessity, use morphologic criteria for
have acute, subacute, and chronic phases with fairly spe- classification at present. Although most inflammatory condi-
ciflc histologic correlates (Fig. 1-9). Therefore, it is impor- tions can be categorized into one of the major reaction patterns,
tant to consider the varying histologic appearances that there are inevitable dermatitides that show overlapping features
can occur ove.r the life span of a skin lesion. and some that defy classification. Some inflammatory condi-
2. The severity of the reaction. For inflammatory lesions incited tions may be sampled either too early or too late in their evolu-
by external or internal antigenic reaction, there is a spectrum tion to be diagnostic. Inflammatory diseases are dynamic, and
of responses ranging from mild to severe (Fig. 1-10). knowledge of the point in time when the dermatitis is sampled
FIGURE 1·9 Spongiotic dermatitis, acute versus chronic changes. Spongiosis with incipient spongiotic vesiculation in early
lesions of allergic contact dermatitis (left) and spongiosis combined with irregular acanthosis, scale-crust, hypergranulosis, and
dermal fibrosis in longer standing lesions of the same disease (right).
Chapter 1 INTRODUCTION TO MICROSCOPIC INTERPRETATION 9
FIGURE 1-10 Interface dermatrtrs, mild to severe. Erythema multiforme in an early lesion shows vacuolar alteration in the basal
layer with few necrotic keratinocytes and few intraepidermal lymphocytes (left). A fully developed or severe expression of the
disease shows numerous necrotic keratinocytes and, eventually ful I thickness necrosis of the epidermis (right).
is critical to optimal microscopic interpretation. The histopa- Interface dermatitides may be further $Ubclassified
thologist should strive to assess specimens with the "fourth according to the density and pattern of the inflammatory
dimension" of time always kept in mind. cell infiltrate in the papillary denni$ a$ (1) the vacuolar or
As mentioned previously, one initially attempts to ascertain cell-poor type, based on perivascular or patchy infiltrates in
whether the epidermis shows one of the following major reac- the papillary dermis or (2) the lichenoid or cell-rich type,
tion patterns or is uninvolved by the inflammatory process. which shows a dense bandlike infiltrate that fills the papil-
lary dermis (Table 1-2). As with all inflammatory processes,
Inflammatory Reaction Patterns of the Epidermis interface dermatitides also may be characterized according
Sponglotlc Dermatitis. "Spongiotic dermatitis'" refers specifi- to their severity (Fig. 1-10, illustrating erythema multiforme)
cally to the presence of spongiosis or intercellular edema that or their stage of evolution as acute or early stage, subacute or
stretches apart keratinocytes and sometimes results in the for- developed, or chronic or late stage. Certain diseases are pro-
mation of intraepidermal vesicles. Spongiosis is often variable, totypic of the two patterns of interface dermatitis mentioned
multifocal, and accompanied by intracellular edema and exo- earlier. Erythema multiforme, many drug eruptions, viral
cytosis of inflammatory cells. The disease process is dynamic exanthems, and connective tissue diseases result in a vacuo-
and in general has been categorized according to morphologic lar pattern of interface dermatitis. On the other hand, lichen
features correlating with the stages of its life history: (1) acute, planus, lichenoid drug eruptions, lichen planus-like kerato-
(2) subacute, and (3) chronic (Fig.1-9). Other alterations, such sis, and "halo" nevus are associated with lichenoid patterns
as granulocyte infiltration of the epidermis (eg, eosinophilic or of inflammation.
neutrophilic spongiosis) or involvement of skin appendages Psoriasiform Dermatitis. "Psoriasiform. dermatitis" refers to
(eg, follicular spongiosls). may be observed.
a characteristic pattern of epidermal hyperplasia typified by
Spongiosis is a relatively nonspeclfic morphologic altera- elongation of the epidermal rete ridges (Fig. 1-11). In general,
tion observed in a wide variety of conditions (see Table 1-1).
the topography of the epidermal surface is unaffected-ie,
It is perhaps the most characteristic of the group of conditions it remains essentially flat-topped. This pattern of epidermal
referred to as "eczematous dermatitis." These disorders include
alteration may be further described as either regular or irregu-
endogenous or atopic dermatitis, contact allergic and irritant
lar. Regular psoriasiform hyperplasia, as the name suggests,
dermatitis, and nummular dermatitis. Other common dermati-
indicates elongated epidermal rete ridges of fairly uniform
tides showing spongiosis include seborrheic dermatitis, spongi-
length and thickness and is typical of psoriasis in a well-developed
otic drug eruptions, and some primary bullous conditions.
stage. This morphologic feature is accompanied by a num-
Interface Dermatitis. "'Interface dermatitis"' refers to a mor- ber of other histologic alterations notable in psoriasis: broad
phologic alteration at the junction or interface between the epi- zones of parakeratosis, absence of the granular layer, exo-
dermis (or epithelium) and dermis. Spedfically, one observes cytosis of neutrophils, pallor of keratinocytes (intracellular
vacuolization (vacuoles or discrete clear spaces) either within edema), thinning of the epidermis above the dermal papillae,
basilar keratinocytes or within the basement membrane zone. prominent dilated and tortuOU$ papillary dermal microves-
This reaction pattern is often accompanied by a number of sels, and papillary dermal edema. Irregular psoriasiform epi-
other alterations present to variable extent: individually dysker- dermal hyperplasia may be observed in psoriasis but typifies
atotic keratinocytes (which are probably apoptotic cells), dis- other processes more commonly, such as chronic eczematous
ruption of orderly keratinocytic maturation to the surface, and dermatitis, lichen simplex chronicus (Fig. 1-11), or mycosis
clefts resulting from coalescence of vacuoles. fungoides (Table 1-3).
10 DERMATOPATHOLOGY
FIGURE 1·11 Psoriasiform dermatitis. Psoriasis shows hypericeratosis with thinned granular layer and elongation of the rete
ridges (left) together with parakeratosis that contains neutrophils in staggered fashion. Irregular psoriasiform hyperplasia
can be seen due to mechanical irritation in lichen simplex chronicus (right), here combined with hypergranulosis, compact
orthokeratosis, and vertically oriented thickened collagen bundles in the papillary dermis.
Overlapping and Distinctive Patterns. When two or more of epidermal hyperplasia. and variable spongi.otic and inter-
the patterns of epidermal alteration are noted, the predominant face alteration. Depending on which of these features might
reaction pattern generally should be used as the basis for cate- predominate, various dermatitides in this group also might be
gorization, or one of the variants below ifclinically compatible. classified as a subacute spongiotic, psoriasifonn, or interface
dermatitis, as below.
Pityriosiform Dermatitis. A small but important group of
These conditions generally include pity.riasis rosea. pityriasis
inflammatory dermatitides shows a constellation of epider-
lichenoides, sebo.rrheic dermatitis, eruptive or guttate psoriasis,
mal changes that include focal or spotty parakeratosis. slight
pre- or early mycosis fungoides lesions (parapsoriasis), some
drug eruptions, subacute eczematous dermatitis, pityriasis
rub.ra pilaris, and superfi.dal fungal infections.
TABLE 1-3 Psoriasiform Dermatitis
Overlapping Reaction Pattems. The four patterns listed below
Psoriasis emphasize the prominence of two or more morphologic altera-
Reactive arthritis tions. Particular patterns often correlate with particular disease
Subacute to chronic eczematous dermatitis processes. For example. spongiotic psoriasiform dermatitis is
a typical pattern associated with chronic active eczematous
Seborrheic dermatitis
dermatitis.
Lichen simplex chronicus
1. Spongiotic psoriasiform dermatitis
Pityriasis rubra pilaris
2. Spongiotic interface dermatitis
Pa rapsoriasis 3. Spongiotic psoriasiform. interface dermatitis
Mycosis fungoides 4. Psoriasiform. interface dermatitis
Psoriasiform drug eruption
Characterization of the Inflammatory Process in the Dermis
Erythroderma
After one has examined the epidermis for morphologic altera-
Candidiasis
tion, one proceeds to evaluate the inflammatory process in the
Secondary syphilis dermis (and subcutis and fascia, as the case may be). A highly
Inflammatory linear verrucous epidermal nevus (ILVEN) signiflcant clue to pathogenesis is whether recognizable vascu-
Scabies lar injury is present or absent.
Lamellar ichthyosis Absence of Vascular Injury. The pattern, depth, density, and
Clear cell acanthoma composition of the inflammatory cell infiltrate are assessed,
with patterns classified as lichenoid, perivascular (Fig. 1-13),
Pellagra
periadnenl, interstitial (infiltrating collagen bundles). nod-
Acrodermatitis enteropathica ular (Fig. 1-14). or diffuse, when occupying the entire der-
Migratory necrolytic erythema mis (Fig. 1-15. Fig. 1-16 and Table 1-6). Regarding depth of
Bazex syndrome infiltration. drug eruptions and viral exanthems often show
superficial perivascular involvement only, whereas conditions
12 DERMATOPATHOLOGY
FIGURE 1-12 Vesfcular and bullous dermatitis. lntraepidennal vesiculation in herpes virus infection (upper left) shows
acantholysis, necrosis, and ballooning of keratinocytes and some multinucleated epithelial cells with steel gray nuclei (upper
right). Bullous pemphigoid is a subepidermal blistering disease (lower left) with numerous eosinophils in the dermal infiltrate and
in the blister (lower right).
such as lupus erythematosus, polymorphous light eruption, would suggest the sarcoidal granulomatous reaction pattern
and se<:<>ndary syphilis are prone to involvement of the deep (see Granulomatous Reaction Patterns, Fig. 1-16).
dermal vascular plexus (ie, a superficial and deep perivascular Presence of vascular injury. •vascular injwY° refers to a spec-
pattern). Deep infiltrates also may be indicative of a systemic trum. of morphologic alterations ranging from endothelial
disease process, as in lupus or secondary syphilis. Infiltrate perturbation or activation to frank ftbrinoid necrosis and the
density is usually assessed in rather subjective terms, such as presence of inflammation for an interpretation of vasculitis
sparse, moderate, or dense. However, recognizing the density of (Table 1-7, Fig. 1-17). These changes may be primary or se<:-
an infiltrate has relevance for particular disease processes, such ondary (a distinction not always easily made). In assessing
as acute urtlcaria (which is sparse), figurate erytb.ema (which vascular injury, a number of parameters must be considered.
is moderately dense), and cutaneous lymphoid hyperplasia or
and these include caliber and type of vessels involved. degree
lymphoma (which tends to be dense). of vascular injury (as already mentioned), the composition and
The cellular infiltrates in the dermis commonly are com- density of the cellular infiltrate, and the presence or absence of
posed of lymphocytes, possibly with varying admixtures of such factors as antineutrophil cytoplasmic antibodies (ANCA).
other cell types, including monocytes/macrophages (histiocytes),
eosinophils, neutrophils. plasma cells, and mast cells. The par- Granulomatous Reaction Patterns. The essential definition of
ticular cellular composition often has diagnostic significance, a granuloma is a drcwnsaibed aggregate of macrophages. The
particularly when integrated with infiltrate pattern, depth, and cytologic characteristics of such cells vary from mononuclear
density. Thus a sparse superficial perivascular infiltrate con- cells with abundant pale, vacuolated, or lipi.dized cytoplasm to
taining lymphocytes and eosinophils (and often neutrophils) cells with plentiful pink cytoplasm, resembling epithelial cells
would suggest urticaria. Infiltrates showing the same cell types and hence the term •epitb.elioid" cells (Fig. 1-6). Multinucle-
but having moderate density nt the broad category of allergic ated giant cells are commonly present and are generally one of
hypersensitivity reactions. Whereas circumscribed superficial two types: foreign-body and Langhans giant cells. Granulomas
and deep perivascular aggregates of epithelioid macrophages often contain a variable admixture of other cell types, such as
Chapter 1 INTRODUCTION TO MICROSCOPIC INTERPRETATION 13
TABLE 1-4 lntraepidermal Vesicular and Pustular TABLE 1-5 Subepidermal Vesicular Dermatitis
Dermatitis
Subepidermal Blisters with Little Inflammation
lntracorneal and Subcorneal Vesicles and Pustules Epidermolysis bullosa
Impetigo Porphyria
Staphylococcal "scalded skin" syndrome Pseudoporphyria
Superficial fungal infection Bullous pemphigoid (cell-poor type)
Pemphigus foliaceus Burns
Pemphigus erythematosus Toxic epidermal necrolysis
Subcorneal pustular dermatosis Bullae associated with diabetes
Infantile acropustulosis Blisters overlying scars
Erythema toxicum Bullous amyloidosis
Transient neonatal pustular melanosis
Subepidermal Blisters with Lymphocytes
Miliaria crystallina
Erythema multiforme
lntraepidermal Vesicles and Pustules
Fixed drug eruption
Spongiotic vesicles Lichen planus pemphigoides
Viral vesicles Polymorphous light eruption
Palmoplantar pustulosis Bullous mycosis fungoides
Friction blister Bullous fungal infections
Epidermolysis bullosa
Subepidermal Blisters with Eosinophils
Bullous pemphigoid
lymphocytes, plasma cells, neutrophils, and mast cells. Poorly Epidermolysis bullosa acquisita
defined granulomatous infiltrates often are referred to as "gran- Pemphigoid gestationis
ulomatous inflammation." Granulomas may be classified in a
Arthropod bites
number of ways, such as infectious or noninfectious or by mor-
phologic features, acknowledging that there is considerable Drug reactions
overlap among many of these entities. A generally accepted Subepidermal Blisters with Neutrophils
scheme is as follows (Fig. 1-16): (1) sarcoidal or epithelioid
cell granulomas, (2) tuberculoid or caseating granulomas, Dermatitis herpetiformis
(3) foreign-body granulomas, (4) suppurative granulomas, (5) Linear lgA bullous dermatosis
palisading/necrobiotic granulomas, and (6) xanthogranulomas. Cicatricial pemphigoid and localized
In general, these reaction patterns are nonspecific and should cicatricial pemphigoid
prompt a differential diagnosis and systematic evaluation, as
Pustular vasculitis
discussed in more detail in Chap. 6 (see Fig. 1-6}.
Bullous lupus erythematosus
Disorders of Skin Appendages. The skin appendages, princi-
pally the hair follicle and the eccrine sweat apparatus, may show Sweet syndrome
primary inflammatory involvement. Epidermolysis bullosa acquisita
Disorders of the Hair Follicle. Folliculitis is categorized as to Erysipelas
whether it is infectious or noninfectious and according to Bullous urticaria
its depth: superficial only or superficial and deep. Acne is an Subepidermal Blisters with Mast Cells
extremely common form of folliculitis that has a multifactorial
basis, for example. The hair follicle also may show a peculiar Bullous mastocytosis
reaction-follicular mucinosis-which may be associated with a Miscellaneous Blistering Diseases
number ofprocesses, including mycosis fungoides and inflamma-
tory conditions such as arthropod bites and lupus erythematosus. Drug-overdose-related bullae
"Alopecia" histologically refers to an overall reduction in PUVA•-induced bulla
the number of terminal anagen hair follicles, which may be Etretinate-induced bullae
reversible or irreversible. Operationally (and perhaps simplis-
tically), alopecia may be classified as nonscarring or scarring. •Psoralen (P) and ultraviolet A (UVA) therapy.
Nonscarring alopecias may result from any number of factors
interrupting the hair growth cycle, whether inflammatory, as in
the case of alopecia areata, or noninflammatory, as in androge-
netic alopecia or telogen effluvium. Scarring alopecia follows
14 DERMATOPATHOLOGY
FIGURE 1·13 Perivascular (and interstitial) dermatitis without epidermal changes. Relatively nonspecific features of a mild
perivascular lymphocytic infiltrate only (upper left) with few plasma cells (upper right) in early cutaneous borreliosis (erythema
migrans). Infiltrates are not only perivascular but also interstitial in erysipelas (lower left), interstitial neutrophils and extravasated
erythrocytes are numerous (lower right).
FIGURE 1·14 Nodular dennal infiltrate due to ruptgre of a folliculitis in rosacea (left). Infiltrates show a mixture of
neutrophils, lymphocytes, and macrophages indicating a granulomatous variant of rosacea.
Chaptu 1 INTRODUCTION TO MICROSCOPIC INTERPRETATION 15
FIGURE 1·15 Diffuse dennal infiltrate {left) with mixed composition {right) induding neutrophils, nudear dust of
neutrophils, eosinophils, lymphocytes and plasma cells in granuloma faciale.
FIGURE 1-16 Different types of granulornu. Tuberculoid granuloma (upper left}: Aggregations of macrophages with
lymphocytic infiltrate$ at the periphery in cutaneou$ leishmaniasis; Insert shows amastigotes within macrophages. Sarcoidal
granuloma (upper right): Aggregations of macrophages with very few lymphocytes at the periphery in sarcoidosis. Suppurative
granuloma (lower left): Aggregation of neutrophils surrounded by macrophages in atypical mycobacteriosis; Insert shows acid
fast bacilli with Ziehl-Neelson stain. Xanthogranuloma (lower right): Diffuse granulomatous infitrates with scattered vacuolated
("foamy"} macrophages in necrobiotic xanthogranuloma: Insert shows foamy macrophages, some multinucleate dose up.
16 DERMATOPATHOLOGY
TABLE 1-6 Dermal Inflammatory Infiltrates without TABLE 1-7 Vasculitis and Related Disorders
Vascular Injury
Vasculopathy
Superficial or Superficial and Deep Perivascular
Small-Vessel Vasculitis
Infiltrates*
Neutroph ilidleukocytoclastic vascu Iitis
Urticaria
Lymphocytic vasculitis
Urticarial reactions
Granulomatous vasculitis
Viral exanthems
Medium-Sized Vessel Vasculitis
Drug eruptions
Gyrate/figurate erythemas
Lupus erythematosus factitial disease, or physical injury. Adequate sampling and the
Polymorphous light eruption stage of disease when the biopsy is taken will influence the mor-
Photosensitive eruptions phologic findings observed. The reaction pattern of adipose tis-
sue to injury is rather limited. Initially one observes an influx of
Chilblains/perniosis
neutrophils (erythema nodosum pattern, Fig. 1-18), followed
Leprosy (indeterminate) by mononuclear cells (lymphocytes and macrophages), and
Syphilis finally, reparative fibrosis, depending on the nature and sever-
Borreliosis ity of the insult.
Leukemia
Proliferative or neoplastic conditions
Urticaria pigmentosa
A large category of conditions encompasses hyperplasias,
Nodular Infiltrates* hamartomas, and benign and malignant neoplasms involving
Arthropod bite reactions the epidermis, melanocytes, skin appendages, dermis, subcutis,
and hematopoietic cells in the skin.
Cutaneous lymphoid hyperplasia
Histiocytic infiltrates Noninflammatory conditions
Neutrophilic dermatoses In the absence of an obvious inflammatory dermatitis or pro-
Lymphoma liferative/neoplastic process, one must proceed along the algo-
rithm considering noninflammatory conditions. Histologic
Diffuse Infiltrates*
alterations may suggest "normal skinn and may include the
Reactive infiltrates so-called invisible dermatoses (Table 1-8).8 As already out-
Leukemia lined earlier, one generally must resort to systematic study of
Lymphoma the specimen beginning with the stratum corneum or perhaps
with the subcutis in the reverse order. It goes without saying
Histiocytic infiltrates
that the histopathologist should have some understanding of
Mast cell infiltrates the regional microanatomy and age-related variations of skin
in order to know what is within normal limits.
•Further classified according to cell types present, such as
lymphocytes, eosinophils, neutrophils, and so on.
Alterations of Stratum Corneum and Epidermis
The stratum corneum is studied for subtle abnormalities
a wide variety of processes, such as infective folliculitis, lupus such as parakeratosis, hyperkeratosis, and fungal elements.
erythematosus, lichen planus, or traumatic injury. The epidermis is inspected for acanthosis, atrophy, subtle
Disorders of the Sweat Apparatus. The eccrine duct may be alterations suggesting an inflammatory or vesicular reaction,
involved primarily in an inflammatory reaction termed "miliarian alterations of the granular layer as in ichthyosiform dermati-
and categorized according to depth of involvement as (superfi- tides, peculiar processes such as epidermolytic hyperkeratosis,
cial) miliaria crystallina, miliaria rubra, and miliaria profunda. and finally, pigmentary alterations associated with hypo- and
Hidradenitis refers to an inflammatory disorder involving the hyperpigmentation.
sweat coil as in neutrophilic eccrine hidradenitis, which may be
Alterations of Papillary Dermis
infectious or noninfectious.
The papillary dermis is then studied for alterations such as
Panniculitis. The primary focus of inflammation may be in the deposition of amyloid, hyalinization of vessels as in porphyria,
subcutaneous fat, fascia, or both. Although traditionally pan- and incontinence of melanin (melanin-laden macrophages in
niculitis has been classified as septal or lobular, in fact, in most papillary dermis).
instances the inflammatory process is both septal and lobular,
often spreading from the septae. Particular factors that should Alterations of Reticular Dermis
be considered when evaluating panniculitis include presence The reticular dermis is examined systematically for alterations of
or absence of infection, vascular injury, cold-related injury, collagen as in morphealscleroderma or scleredema, thickening
Chaptu 1 INTRODUCTION TO MICROSCOPIC INTERPRETATION 17
FIGURE 1-17 Vascular injury. Fibrin in the vessel wall of a small vessel together with infiltrates of neutrophils and nuclear
dust in leukocytoclastic vasculitis (upper left). Thrombus in 1he lumen of a small vessel in the subcutis in a coagulopathy (upper
right). Fibrin and inflammatory infiltrates in the vessel wall of a medium-sized artery in polyarteritis nodosa (lower left). Calcium
deposition in a vessel wall in calciphylaxis (lower left).
FIGURE 1-18 Panniculitia. Prominent alteration of the subcutaneous fat in erythema nodosum. Rbrosis of the septae and
infiltrates of macrophages with multinucleated giant cells, lymphocytes and neutrophils are present in the broadened septae.
18 DERMATOPATHOLOGY
or atrophy of the reticular dermis, alterations of elastic fibers, inflammatory cell type) due to the incorporation of automated
and deposition of materials such as mucin or amyloid. feature detection algorithms.
signatures13 and next-generation sequencing methods14 show- 2. Duncan LM, Berwick M, Bruijn JA, et al. Histopathologic recognition
ing promise. and grading of dysplastic melanocytic nevi: an interobserver agree-
ment study. J Invest DermatoL 1993;100(3):S318-21.
Techniques to further characterize 3. Ackerman AB. Differentiation of benign from malignant neoplasms
by silliouette. Am J Dermatopathol. 1989;11 (4) :297-300.
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As described above, patterns of infiltration can help in defining digital images versus traditional glass slides in the detection of com-
the differential diagnosis(es) based on morphology alone. The mon microscopic features seen in dermatitis. I Pathol Informatics.
introduction of digital slide imaging and feature analysis may 2016;7:30.
5. Ferrara G, Di Blasi A, Zalaudek I, et al Regarding the algorithm for
lead over time to more precise enumeration of inflammatory
the diagnosis of early mycosis fungoides proposed by the International
cell types/ but currently routine histology is sufficient to gener- Society for Cutaneous Lymphomas: suggestions from routine histopa-
ate the appropriate differential diagnosis. thology practice. JCutaneous Pathol. 2008;35(6):549-553.
Adjunct immunohistochemical characterization is most 6. Wong C, Peters M, Tilburt J, et al. Dermatopathologists' opinions
useful for dense dermal lymphoid infiltrates, with CD20+ about the quality of clinical information in the skin biopsy requisition
B-cell-rich infiltrates warranting consideration of B-cell lym- form and the skin biopsy care process: a semiqualitative assessment
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7. Trotter MJ, Au S, Naert KA. Practical strategies to improve the clin-
mixed or predominantly CD3+ T-cell infiltrates may support
ical utility of the dermatopathology report Archiv Path & Lab Med.
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adnexal infiltration, CD4 and CDS immunostains can often 9. Onega T, Reisch LM, Frederick PD, et al. Use of digital whole slide
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1. Ackerman A, Boer A, Bennin B. Gottlieb G. Histologic Diagnosis of 2014;415;1-5.
Inflammatory Skin Diseases: A Method of Pattern Analysis. 3rd ed.
Philadelphia, PA: Lea & Febiger; 2005.
Spongiotic
Dermatitis CHAPTER 2
Preeti Jhorar • Michael Murphy • Jane M. Grant-Keis
Spongiotic Dermatitis
1
Proliferation of the
1
Superficial perivascular
1
Supedicial and deep Wedge-llhapc4
1 1
Supedicial perivascular
mpetficial vascular l~ocylic infiltrate with perivascular lymphoid infiltrate, superficial and deep lympbtlid infiltrate and
plexus, thickCIJai hcmmrhagc md/or tightly cuffed "ccat-slcc:vc" perivascular lymphoid inlnepidamal or intra-
vessel walls, variable bemosiderin- around ves11el: infiltrates with eosinophilll: corneal neutrophils:
amount of lymphocytic Wien IIlllCrophages: Figurate erytbemas Hypenenaitivity reaction Seborrbeic dermatitis
infiltrate, hemorrhage, Pigmentm purpuric to arthropod assaults Early or trcatm psoriasis
and hcmosiderin-laden
Dyskcratotic cells:
dc:rmatosis (eczematous Sneddon-Wtlldnaon
macrophages: variant ofDollC8S and Polymorphous light rcactioo disease
Photodrugn:action
Chronic Italia dermatitis Kapetanalris) Contact dermatitia
Many dyskttatotic cells and
Acromiiodermatitis (esp. irritant)
kttatinocytic atypia:
Phototoxic reaction
1
Superficial perivascular
l
Superficial perivascular
l
Superficial perivascular lymphoid
l
With intracomeal hypbal fOIIDll:
lymphoid infiltrate with lymphocytic infiltrate infiltrate with or without eosinophils: Dennatophytosis
or without cosinopbils without eosinophih: Drug hypersemitivity rcactioo
and large, well-formed
With intracomeal gram-positive
Pityriaaia lichenoi.dcs chrooica Atopic dermatitis cocx:i:
intniepidennal vesicles:
Polymorphous eruption of pn!gnmcy Nummular dermatitis Impetigo
Pompholyx
Gianotti-Crosti 1yndrome Pityriaaia rosea (eapecially inllllllllillllory variant) With plasma cells and
Allergic contact dermatilis
Early myc01il fungoide& Grover's dUeasc: (spongiotic variant) endothelial cell swelling:
Dyllllidrotic ccuma
Idn:action Secondary syphilil
FIGURE 2..3 Acute sponglotrc dennatrtrs. Marked epidermal FIGURE 2-6 Subacvte spongfotlc dermatitis. Parakeratosis,
intercellular edema, micro and macrovesicles, and superficial scale crust, slight epidermal acanthosis, intercellular
perivascular lymphocytic and eosinophilic infiltrate. There is spongiosis, and superficial perivascular lymphohistiocytic
focal parakeratosis. infiltrate.
FIGURE 2-4 Subacute spongiotic dermatitis. Parakeratosis, FIGURE 2-7 Chronfc sponglotlc dennattds. Compact
slight epidermal acanthosis, minimal spongiosis, and hyperkeratosis, parakeratosis, slight hypergranulosis,
occasional lymphocyte exocytosis. epidermal acanthosis, and spongiosis.
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have a perfect right to do as they please in such matters. You must
not take it up personally.”
The speech was not judicious. Cicely raised her head proudly;
there came an unusual light into the soft eyes, the lines about the
gentle mouth grew hard.
“A perfect right,” she repeated. “Yes, of course they have a perfect
right to give a ball whenever they please. But they have no right to
expect me to go to it. I am engaged to their son certainly, but if they
disregard my feelings and consider me no more than a stranger, it
leaves me free to behave like one. How could I wish to go to a ball?
Think of what sorrow we have had so lately—think of my father’s
state oh! mother, it is most inconsiderate.”
“My dear, you are hardly reasonable,” said Mrs. Methvyn. “You
are very honest, Cicely,” she went on. “Tell me, dear, is it not partly
that you are hurt at not having been consulted about it at all, at not
having been asked if the idea of such a thing was pleasant to you?”
Cicely was silent for a little. Then she said slowly, “Yes, I think it is
partly that. But I don’t think it is from any small or mean feeling of
vexation at not being consulted. It is that it seems to me that Trevor
is different.”
“Wait till to-morrow,” said Mrs. Methvyn sagely. What she had said
had done some good. It inclined Cicely to restrain her first
vehemence of feeling, to receive more gently Mr. Fawcett’s
explanation of what had led to this unexpected piece of dissipation. It
sounded simple enough when, as Cicely expected, he came the next
morning to talk it over with her. They had been speaking about balls,
he said, one evening at Eastbourne, and Geneviève, who (though in
some mysterious manner she had learnt to dance) had never been
at any entertainment of so “wholly worldly” a kind, had expressed
with girlish eagerness her intense wish to assister at a real ball. Half
in joke, half in earnest, the idea had been mooted; Sir Thomas, who
Trevor declared had altogether lost his heart to his pretty visitor, had
taken it up and promised to open the ball with her himself, “and,”
said Mr. Fawcett in conclusion, “the day was fixed for the twentieth of
October, my birthday, you know, Cicely.”
“Yes,” said Cicely, “I remember.”
Her tone of voice aroused Trevor’s misgivings.
“Don’t you like the idea of a ball, Cicely?” he asked. “I am sure
you used to like dancing, especially in the country. And I thought you
would have been glad for Geneviève to be pleased.”
“Her notions of pleasure and mine differ,” said Cicely coldly, “if she
can find it in amusing herself in a way her parents would disapprove
of.”
“Rubbish,” said Mr. Fawcett. “What can they know about it? They
would not expect Geneviève to behave differently from other people.
She is ‘at Rome’ now, and they must take the consequences of
sending her there.”
“I am not dictating anything to Geneviève,” said Cicely more
gently; “she must judge for herself. As to my own feeling about it, I
confess to you, Trevor, I would have much preferred not taking part
in anything of the kind at present, but—”
“But what?” said Mr. Fawcett.
“If you wish it, I will try to dismiss the feeling I have,” she
answered.
“If I wish it. Cicely, you speak as if I were an unfeeling tyrant. It is
not fair to me, upon my soul it isn’t,” said Trevor, working himself up
into vexation. “No one felt more for you than I did last spring, but you
cannot shut yourself up for ever.”
“It is not only that,” said Cicely. “I have other feelings—my father’s
state of health, my having to leave him so soon—all these things
make me sad. But I dare say it is wrong to feel so. Dear Trevor, don’t
be vexed with me. I will try and enter into it cheerfully. If I had been
with you and could have talked it over with you and your mother
before, it would have been all right.”
“I wish you had been with us when it was first proposed. You don’t
know how I wished for you at Eastbourne, Cicely,” exclaimed Trevor.
Cicely looked up at him affectionately. For the moment their old,
happy relations seemed to have returned—the vague, painful feeling
“that Trevor was different,” which of late had so often troubled her,
melted away. And for the rest of that day Cicely’s brow looked clear,
and her eyes had a smile in them.
But Geneviève’s brilliant spirits seemed already to have received
a check. She was tired, she told Cicely, she thought one always felt
so the day after a railway journey more than at the time.
“Are you too tired to talk about what you will wear at the Lingthurst
ball?” said Cicely brightly. “Mother wants us to have very pretty
dresses, and I am going to order them from town; so we must have a
grand consultation, Geneviève.”
Geneviève looked up in amazement.
“I thought you were angry about it—I almost thought you would
say you would not go,” she exclaimed.
Cicely was silent for a moment. Then she said quietly,—“It is true I
was surprised, and not pleased at the idea of it last night. But I think
it was unreasonable of me, and I am sorry for having chilled your
pleasure in it.”
“You are very good, Cicely,” said Geneviève. “I wish I were as
good as you.”
She sighed. Cicely looked at her with some surprise.
“You are not to go off into a fit of low spirits, Geneviève,” she said,
in a rallying tone. “I am not good when I am cross—the least I can do
is to say I am sorry, isn’t it? But if you look miserable it will be like a
reproach to me. I was so pleased to see you so bright and merry last
night. Now tell me about your dress. What would you like it to be?”
“White,” said Geneviève decidedly. “It is as it were my first ball,
you see, my cousin.”
“Yes,” said Cicely drily. “I suppose you did not go to balls at
Hivèritz?”
“No,” replied Geneviève, in the most matter-of-fact tone. “Papa
being a pasteur, you understand, it would hardly have been
convenable that I should go to balls there.”
“And what will your parents say to your going here?” inquired
Cicely.
“Oh! I don’t think I shall say anything about it,” answered
Geneviève carelessly. “Not that I think mamma would object—she
has placed me under the care of my aunt—it is not for me to dictate
to your mother, Cicely.”
Cicely did not contradict her, and Geneviève proceeded to
discuss the important question of her dress. She warmed into
enthusiasm on the subject, quite astonishing her cousin by her
display of millinery lore and perfect acquaintance with the
requirements of the occasion.
“I can hardly believe you have never been at balls, and all sorts of
things of the kind, Geneviève,” she exclaimed at last. “Where did you
learn to dance?”
“There was a class at the pension where I went. I used to watch
them and then try by myself afterwards,” said Geneviève. “It is quite
simple. Mr. Fawcett says I dance very well.”
“Trevor!” exclaimed Cicely. “How does he know?”
“Oh! we only tried once—là-bas—at Eastbourne, I mean, when
the band was playing a waltz before our windows,” said Geneviève
hastily. “Tell me, Cicely,” she went on quickly, “who will there be at
the Lingthurst ball that I shall know. Will Mr. Guildford be there?”
“No, I am sure he will not,” replied Cicely. “He has other things to
do.”
“But he comes here very often. He can not be very busy,” pursued
Geneviève. “My aunt tells me he has been here three—four times in
the week.”
“He doesn’t come here for pleasure—it is perfectly different,”
answered Cicely coldly.
“Is it? Ah! yes, I see. He comes here but as my uncle’s doctor,”
said Geneviève so innocently that Cicely felt ashamed of the slight
feeling of annoyance which her cousin’s remarks aroused in her.
“I wonder if she has heard Trevor speak of Mr. Guildford in that
foolish way,” she thought to herself. “Trevor should be careful.
Geneviève does not understand—she will be treating Mr. Guildford
as if he were beneath her.”
But her fears were misplaced. When Mr. Guildford came the next
day, Geneviève made herself as charming as ever. She smiled and
blushed more than she talked, it is true; but once or twice Cicely
caught Mr. Guildford’s eyes resting upon her in a way which awoke a
new feeling in her mind. “Does he really care for her?” she said to
herself uneasily. “He, so clever and good. Is she worthy of it?”
She felt more than ever that she could not understand Geneviève.
There were times at which it seemed to her that a creature more
artless and ingenuous could not exist—that the feeling of
bewilderment about her must arise entirely from her own in ability to
be carelessly, childishly transparent like this sunny little fairy. Then
again a sudden glimpse of something very like calculating
selfishness on Geneviève’s part would startle her into perplexity
again, and then would follow a fit of disgust at her own
suspiciousness.
“Do you understand Geneviève, Trevor?” she asked Mr. Fawcett
one day. It was the very day before the ball. They had been at
luncheon at Lingthurst, discussing and admiring the all but
completed arrangements, and Trevor had walked home with Cicely.
Geneviève had been invited to come with them, but for some reason
that Cicely was at a loss to explain, had refused to do so, and had
driven home with her aunt.
“Do you understand her?” Miss Methvyn repeated, for Mr. Fawcett
had not seemed to hear her question the first time.
Trevor started. “What are you saying, Cicely?” he exclaimed. “Do I
understand Geneviève? Of course, I do. You are always diving into
unknown depths or soaring into the clouds, my dear child. Please
remember that other people find it fatiguing. You must be at a loss
for a subject of speculation if you are going to make one of poor
Geneviève—she is just a sweet, simple little creature, very
affectionate, and not very wise, and perhaps a little vain; which is
certainly excusable. There is not much to understand about her.”
“Is that it?” said Cicely thoughtfully. She had listened attentively to
what Trevor said, looking up into his face with a questioning,
somewhat anxious expression in her eyes. Somehow it annoyed
Trevor. He began kicking the pebbles on the path impatiently. But
just for the moment, Cicely was too intent on what she was saying to
observe his irritation.
“I wonder if it is so,” she repeated consideringly. “Sometimes I feel
as if she were perfectly artless and sweet and unselfish. And then
she says and does things that I don’t like, or rather that I don’t
understand. To-day for instance.”
“What did she do to-day?” said Trevor sharply. “I declare Cicely
you are just as bad as other women after all—everlastingly picking
holes in each other—especially if “each other” has the misfortune to
be bewitchingly pretty!”
The sneering tone as well as the unkindness of the speech
wounded Cicely to the quick. She turned her face away, and walked
on without speaking.
“Cicely,” said Mr. Fawcett in a minute or two.
No answer.
“Cicely,” he repeated.
“What, Trevor?” she said gently. Her tone was sad, but nothing
more.
“What are you offended at?” he asked. “I did not in the least mean
to vex you—you might know that—but you take up things so hastily
now. You, who used to be so sweet-tempered.”
His words touched her. Cicely’s conscience was very tender.
“Am I ill-tempered?” she said anxiously. “You never used to think
me so, but perhaps it is true. I don’t understand myself now, it seems
to me, so I should not be hard upon Geneviève.”
“That’s just it,” said Trevor. “You are hard upon her, Cicely, and I
have always thought so. What was it that she did to vex you to-day?”
“I would much rather not speak about it any more,” said Cicely. “It
only makes you think me unkind, and perhaps I am fanciful.”
“No, I won’t think you unkind. Do tell me. I want to know what it
was.”
“It was when we were talking about to-morrow. Something was
said about your dancing first with me, and you said I must certainly
keep half-a-dozen dances for you, as it was so long since we had
had any, Don’t you remember?”
“Well? Yes, I think I do.”
“Geneviève was beside me at the time. When I turned round to
speak to her, she would not answer me. Then all of a sudden she
muttered something about wishing she had never come here. And
when you went away, and I asked her what was the matter, she
began to cry, and accused me of unkindness and selfishness and all
sorts of things. She was just offended at not being made first in
everything. And I have tried to make her happy, Trevor.”
“She is a spoilt child,” said Trevor carelessly, “but you need not
trouble yourself so much about her. When we are married, Cicely,
and she has it all to herself at Greystone, she will be all right, you will
see.”
“Then you do think she dislikes me, Trevor?” said Cicely quickly.
“That is the feeling I don’t understand. She almost seems—I don’t
like saying so—but she almost seems jealous of me.”
Trevor laughed, but his laugh was not hearty.
“Really, Cicely, you must not take things up so seriously,” he said.
His tone was not unkind this time, however. They were close to the
Abbey grounds, and Trevor stopped as if about to turn back.
“I must go home again now, I think,” he said. “Good-bye, Cicely.
You will give me the first dance to-morrow, and half-a-dozen others,
even if Mademoiselle Geneviève is offended, won’t you?”
Cicely smiled. “I think I can brave her displeasure,” she said.
“Good night, Trevor; you won’t come in?”
“I can’t,” he replied. “My mother begged me to come back soon.
Miss Winter and I will be kept at work all the evening, I expect, for
my mother is never satisfied with anything till it has been undone and
then put back again as it was originally. Good night.”
He strode away. Cicely stood watching him for a minute, then
taking the key from her pocket, she unlocked the little door near
which she was standing, and passed through into the park. How
many times she had done so in her life; how far from her thoughts it
was just then that this might be the last time she would pass through
that little old doorway; how seldom any of us think that to even the
commonest and most familiar actions of our daily lives there must
come a “last time!” A last time in many cases not known to be such,
till looked back upon from the other side of some sudden crisis in life,
or sometimes, it must be, from the farther shore of the dark river
itself. And it is well that it should be so. We could make no progress
in our journey were we constantly to realise the infinite pathos
attending every step; we should sink fainting by the way did we
suspect the mines of tragic possibilities over which we are ever
treading.
When Cicely entered the hall she met Geneviève, who was
crossing it on her way to the library.
“Have you come back alone?” she said quickly, when she saw
that there was no one with her cousin.
“Oh! no; Trevor came to the park door with me,” replied Cicely.
“He had to hurry back again. Have you and mother been home
long?”
“Yes, a good while. You have missed some one,” said Geneviève,
“Mr. Guildford has been here.”
“Oh! I am so sorry; I wanted to see him!” exclaimed Cicely. “Why
would he not stay?”
“He saw my uncle,” said Geneviève shortly. “That was what he
came for. I told him where you were; he left no message.”
“I didn’t expect any message,” said Cicely, not quite
understanding Geneviève’s curious tone.
“Yes, you did,” answered the girl bitterly, “or you expected him to
wait for the chance of seeing you. You think you are to be queen of
all—if you are there no one must have a word, a glance! I have said I
loved you, that you were good; but I think not so now. I love you not.
You are cold and proud, and know not what love means, yet you
gain all! And I—I am miserable and alone, and who cares?”
“Geneviève, you must be mad! I do not know, and I do not wish to
know, what you mean. You have yielded to-day to temper till you
have completely lost your reason, that is the only excuse I can make
for you.”
Then Cicely walked quietly across the hall and down the passage
to the library, leaving her cousin standing alone. Geneviève did not
follow her. When Cicely had gone, she ran upstairs to her own room
and threw herself down upon the bed, sobbing bitterly.
Miss Methvyn found her mother in the library.
“Mr. Guildford has been here, Cicely,” said Mrs. Methvyn as she
came in.
“Yes, I know; Geneviève has just told me. I wish I had seen him. I
think he might have waited a few minutes.”
“He said he would; he seemed to want to see you,” said Mrs.
Methvyn. “I told him you would not be out long, and he seemed in no
hurry, and went out into the garden with Geneviève. Then, to my
surprise, in about a quarter of an hour he came in again suddenly
and told me he had just remembered an engagement at Sothernbay,
and that he could not possibly wait any longer. But he is coming
again to-morrow.”
“To-morrow,” repeated Cicely. “Why should he come so soon
again!”
“I don’t quite know,” said her mother. “Cicely,” she went on
tremulously, “I am afraid he does not think your father quite so well.”
“Do you think so, dear mother?” said Cicely. “I hope not. You get
nervous. I wish I had been in.”
“So do I,” said Mrs. Methvyn. “I fancied from his manner that he
would have spoken more openly to you.”
“What did he say? Tell me exactly, mother,” said Cicely. Her voice
sounded calm, but inwardly a sort of icy tremor seemed to have
seized her. She would not tell her mother that even to her eyes a
slight change had been visible in her father for the last day or two;
she had tried to persuade herself that it was “only her fancy;” but she
had longed for Mr Guildford’s next visit with intense though
concealed anxiety. “Do tell me all he said,” she repeated.
“He did not say much. It was before he had seen Geneviève,”
replied Mrs. Methvyn. “After he had been with your father, he came
down here and asked when you would be in. Then he said he
thought your father rather “low” to-day, and that he had been trying
to persuade him not do so much—to get a proper man of business to
manage things, and not to worry himself. I think it is true, and I told
Mr. Guildford I agreed with him. I know Phillip has been annoyed the
last few days by some letters he got.”
“What letters? He never told me about them,” said Cicely.
“You would not have understood them. I do not. I only know they
were about money matters,” replied Mrs. Methvyn vaguely.
“Money matters,” said Cicely. “Oh! he really should not trouble
himself about things of that kind.”
She spoke more cheerfully. There was a certain relief in being
able to name a cause for her father’s depression. And to her happy
experience the expression “money matters” bore no terrible
significance. She was only thankful that his anxiety arose from no
more important cause.
“No; I wish he would not,” sighed Mrs. Methvyn.
“Well, Mr. Guildford will be here to-morrow, and then we can talk it
over with him, and make papa do what we tell him,” said Cicely
brightly.
She was leaving the room when her mother recalled her.
“Cicely,” she said mysteriously, “do you know that there was
something very odd in that young man’s manner this afternoon?”
“How? what do you mean, mother?” replied Cicely. “You speak as
if he were going out of his mind.”
“Nonsense, my dear, you know quite well what I mean,” said Mrs.
Methvyn. “I really do believe he has got something in his head about
Geneviève. It was after he had seen her in the garden that he came
in and said he must go home at once.”
“But why should seeing Geneviève in the garden have made him
say so?” inquired Cicely.
“My dear, how can I tell? When people are in love, there is no
accounting for what they will do. Geneviève may have been cold to
him, or—he is a very modest young man—he may think we should
not approve of it, and may have been afraid of being tempted to say
something. Who can say? I only say that I feel sure he has got
something of the kind in his head.”
Cicely looked grave. “Perhaps he has,” she replied. To herself she
said, “I wonder why, if it is so, it should have made Geneviève so
desperately cross.”—“Mamma,” she added, after a little silence, “I
wish you would do something to oblige me.”
“What, my dear,” said Mrs. Methvyn in surprise.
“Please don’t call Mr. Guildford ‘a very modest young man.’”
CHAPTER X.
FORGIVE ME, AND GOOD-BYE.
IT was the day of the Lingthurst ball. Cicely woke early, and tried to
believe that she was in good spirits, and that her anxiety of the
evening before had been exaggerated and uncalled for. And when
her mother met her with the good news that Colonel Methvyn had
had a calm and undisturbed night, and seemed wonderfully
refreshed by it, the make-believe seemed something very like reality,
and Cicely’s face looked bright enough when she met her cousin in
the breakfast-room to satisfy Geneviève that her ebullition of the
previous day had been forgiven, if not forgotten, or that at least it
was to be tacitly ignored.
Geneviève was excited, but not happy. Some closeness of
observation is, however, required to discriminate between the two
conditions, and this neither of her companions was this morning
sufficiently at leisure to bestow upon her. So, “poor Geneviève is full
of her ball. I hope she will enjoy it,” thought Mrs. Methvyn; and
“Geneviève cannot have meant what she said yesterday. It must just
have been one of her childish little fits of temper, not worth noticing,”
was the decision Cicely arrived at.
“Your father is very anxious for his letters this morning,” said Mrs.
Methvyn, as they were sitting at breakfast. “I hope there will be
nothing wrong in them—nothing to upset him, when he seems so
much better.”
Just as she spoke the letter-bag was brought in. Mrs. Methvyn
opened it.
“Two for you, Cicely,” she said, as she distributed the budget; “one
for Geneviève, three for your father, all business letters I fear.” She
looked at them anxiously. “I wish we could keep them till Mr.
Guildford comes.”
“It would be no use. Papa would be sure to ask for them,” said
Cicely decidedly. “Give them to me, mother; I will take them up to
him myself.”
“Is Mr. Guildford coming to-day?” said Geneviève in surprise, as
her cousin left the room.
“Yes,” said Mrs. Methvyn; “he promised yesterday, when he had
to leave in such a hurry, that he would come again to-day.”
“Oh!” said Geneviève. Then, fancying her aunt looked at her
curiously, “I thought that he was so very busy,” she added
confusedly.
Cicely meanwhile was knocking at her father’s door. Her first tap
was unnoticed. She repeated it.
“Come in,” said Colonel Methvyn’s voice. To Cicely it sounded
very weak and feeble. “Oh! is it you, my dear?” he exclaimed when
he saw her. “I thought it was Barry with the letters.”
“I have brought them, papa,” said Cicely. “But I do so wish you
would not read them yet. They look like business letters, and they
always tire you so.”
She stooped and kissed him. He had had a good night Mrs.
Methvyn had said, but to Cicely’s eyes he looked sadly white and
frail this morning; his voice was tremulous, his hand shook as he
held it out for the letters.
“Give them to me, my dear child. I shall be more comfortable
when I have read them.”
He opened two of them and tossed them aside with indifference.
The third was a longer letter. Colonel Methvyn read it through once—
twice—then folded it up again and put it back carefully into its
envelope with a little sigh. Cicely watched him anxiously.
“Is it all right, papa?” she said. “Nothing to vex you, I mean?”
“Oh! no, it is all right enough,” he answered rather absently.
“Cicely,” he went on, after a little pause, “there will probably be a
telegram for me some time to-day. Don’t think of keeping it from me,
my dear. It would annoy me inexpressibly if you did so. Let it be
brought up at once. Tell your mother so.”
“Very well, papa,” replied Cicely. She leant over him and kissed
him again, then she went quietly downstairs.
Her mother looked up quickly as she re-entered the room.
“I don’t think there is anything particular in papa’s letters,” said
Cicely, in answer to her mother’s unspoken question. “But he says
there may be a telegram some time to-day, and he wishes it taken to
him at once.”
“I hope it won’t come,” said Mrs. Methvyn. “I don’t feel easy about
your father. He is doing far too much. How do you think he is looking
this morning, Cicely?”
“Pretty well,” replied Cicely. “What time do you think Mr. Guildford
will be here, mother?”
“Early—before luncheon, I fancy,” said Mrs. Methvyn. “You will not
be out today when he comes, my dear?”
“Oh! no,” said Cicely. “I wish I knew what time he will be coming,”
she thought to herself, “I would walk part of the way to meet him.”
For since seeing her father her fears had revived. She felt certain
that Mr. Guildford must have thought unfavourably of him the day
before, otherwise he would not be coming again so soon; she felt
restless and unhappy, and longed with intense longing to express
her fears to the only person who could soothe or allay them; the
thought of the ball at Lingthurst grew hourly more distasteful to her.
“If only Geneviève could go alone,” she thought, “and mother and
I stay at home. But, of course, it would give offence—I must go.”
She could settle to none of her usual occupations, and at last she
determined to set off to meet Mr. Guildford. She looked in at the door
of her cousin’s room before going. Geneviève was laying out
betimes her costume for the evening, apparently perfectly happy in
the occupation; she looked up with a bright smile at the sound of
Cicely’s voice.
“Is not the effect of these flowers on the skirt beautiful, my
cousin?” she exclaimed, pointing to the mass of snowy clouds of
gauze that lay on the bed. “I only wish it were time to dress. I am all
impatience to put it on.”
“It is very pretty,” said Cicely kindly. “I am sure it will suit you
beautifully, Geneviève. I am going out for a little,” she went on,
“please tell mamma so if you hear her asking for me. I cannot disturb
her just now. She is in papa’s room. You don’t want to come out this
morning?”
“Oh! no, thank you,” said Geneviève, “I have twenty things to do. I
don’t like the bows they have put on my boots, they make the foot so
broad. I am going to arrange them again.”
“Well, good-bye then,” said Cicely, turning to go. Just then there
came a ring at the front-door bell. It sounded sharp and loud through
the quiet house.
“Who can that be?” exclaimed Geneviève.
“The telegram,” said Cicely. “I must go and see if it is.”
“Stay a moment. I can tell you,” said Geneviève.
One of the windows of the room looked to the front, but the sill
was high and narrow. She drew a chair forward and stepped up on to
it. Cicely watched her in astonishment.
“What are you doing, Geneviève?” she exclaimed. “You can’t see
anything from there. You forget the porch.”
“Ah! but I can,” replied Geneviève triumphantly. She was by this
time mounted on the sill, craning her neck round in a peculiar
fashion. “You forget there is a window in the side of the porch. From
here, when I put my head so, I can see who stands at the door—
voilà! I found this out the first days I was here. Now I see. No, Cicely,
it is not the boy from the station. It is a tall figure, a gentleman. Can it
be Mr. Fawcett?”
She turned round with eager inquiry.
“No,” replied Cicely, “I don’t expect him to-day. Do come down,
Geneviève. It would look so strange if any of them saw you climbing
up there.”
She spoke rather coldly. Geneviève’s conduct jarred upon her.
She only waited till the little lady had accomplished her descent in
safety, and then went downstairs, to satisfy herself of the correctness
of her cousin’s information.
She was not long left in doubt. Parker was coming in search of
her—Mr. Guildford was in the library and had asked for her.
“How kind of him to come so early,” thought Cicely, trying to
believe that no thing but kindness was the motive for such prompt
fulfilment of his promise. “If he were really uneasy about papa, he
would certainly have waited to see me last night,” she said to herself,
as she entered the room; but, nevertheless, she looked strangely
pale, and the tremor in her voice was not quite imperceptible when
Mr. Guildford came forward to meet her. He shook hands somewhat
abruptly. Cicely glanced at his face. He too seemed discomposed;
he looked worn and tired, as if he had not slept all night. A terror
seized Cicely. “Has he come to break it to me? Does he think the
very worst?” were the thoughts that flashed through her mind. She
felt herself beginning to tremble so much that she sat down on the
nearest chair without attempting to speak.
Mr. Guildford did not seem to notice her agitation; he did not look
at her, but kept his eyes fixed upon the table beside which he was
standing.
“He is afraid of looking at me—he cannot make up his mind how
to tell me what he must,” thought Cicely, with a sort of shiver. But the
silent waiting at last grew unendurable; she felt that it must be
broken.
“It is very kind of you to have come so early,” she began. “I cannot
tell you how kind I think it.”
Mr. Guildford turned suddenly. “I came early on purpose,” he said.
“I was so afraid of missing you. But how ill you look, Miss Methvyn,”
he went on hastily. “Is there anything wrong? You look so dreadfully
pale. I am afraid I should not have asked to see you.”
Cicely’s pale lips quivered. “I am quite well,” she whispered.
“There is nothing wrong with me. I shall be all right again directly—
but, Mr. Guildford, I—I know why you have come this morning I know
what you have to tell me. Please don’t hesitate—it is better not. I
shall not be silly—you will see.”
She tried to smile, but hardly succeeded. Mr. Guildford looked at
her in amazement. “You know why I wanted to see you this morning,
Miss Methvyn?” he repeated. “You cannot. It is impossible that—that
you should suspect,” he stopped in confusion.
“I have thought him much less well the last few days,” said Cicely.
“Of course I cannot judge as you can, but still I almost expected you
to tell me you were beginning to lose hope. I knew you would tell me
first.”
“Are you speaking about your father?” said Mr. Guildford. “Did you
think it was on his account I wanted to see you?”
“Yes, of course,” replied Cicely wonderingly. “Is it not so? Do you
not think him much worse?”
“No,” said Mr. Guildford. “I have not thought so. I do not think
Colonel Methvyn quite as well as he was some time ago—he is more
nervous, more easily upset than he used to be; but I see no
important change in him. There is no reason why he should not
remain as well as he is—or even gain ground a little—for years to
come, provided always his mind is kept tranquil. I could not take
upon myself to say how he would stand any severe shock.”
Cicely gazed at him as if she could hardly believe him.
“That is just what you said some months ago,” she exclaimed.
“And you really don’t think him much worse?
“Certainly not. What made you think so?”
“I don’t know. I have not thought him quite well. I fear he has been
worried and troubled, and I have let my fears get the better of me, I
suppose. I felt quite certain that you had come this morning to
prepare me for something dreadful.”
She smiled, but faintly still—the revulsion from terror to renewed
hope was almost too much for her. Mr. Guildford smiled too, but in
his smile there was even less sunshine than in Cicely’s, and in his
voice there was even a touch of bitterness as he replied,
“Something dreadful! Far from it. You will believe me when you
hear what it really is that I want to say to you this morning—” he
paused and took a step or two away from where he had been
standing. Then he came back again to the table, and, lifting a book
that was lying upon it, turned the leaves over idly with his fingers. “I
want you to release me from a promise, Miss Methvyn,” he said at
last.
Cicely looked up in surprise. “What promise? I don’t understand,”
she replied.
“Don’t you remember,” he went on, speaking slowly, but without
looking at her, “don’t you remember that some time ago I promised
you—tacitly or directly I am not sure which, and it does not matter,
the promise was given—that I would not leave this neighbourhood as
long as Colonel Methvyn required me—as long as I felt that I could
be of use to him?”
“Yes,” said Cicely, “I have always depended upon your not doing
so. I don’t remember the exact words, but I felt satisfied that you had
perfectly re-assured me about it, at the time I was afraid of your
going.”
“Yes. I did promise as I said. There is no doubt I did,” said Mr.
Guildford, and it is from this promise I want you to release me.”
“You want to go away! You have got some better position in
prospect!” exclaimed Cicely. “Oh! how unfortunate—can you not
defer going, even for a few months? Papa may be stronger, or Dr.
Farmer may be back; of course, we cannot expect you to sacrifice
your future to us, but I cannot help telling you I am dreadfully sorry. I
was so thankful to hear you say that you do not think papa much
worse, and now, I shall just feel more anxious about him than ever.”
She turned her head away, but Mr. Guildford felt that there were
tears in her eyes.
“You need not—you must not think I would act without regard to
Colonel Methvyn,” said Mr. Guildford hurriedly. “I have heard from Dr.
Farmer—he is not likely to be away very much longer—and in the
meantime I can assure you that the medical man I should
recommend to your father is thoroughly deserving of your
confidence.
“I dare say he is,” said Cicely impatiently. “It is not that that I am
thinking of. I don’t believe any doctor can do much for my father. It is
not doctoring he needs as much as cheering and interesting. That is
what you have done for him—far better than poor old Dr. Farmer
could do. And he will miss you after a while even more than now;
there are reasons—” she hesitated. “Oh! I am dreadfully sorry,” she
repeated, “but of course we cannot expect you to sacrifice your
future. We are only too grateful for what you have done. Forgive me
for seeming so selfish.”
Mr. Guildford did not appear to notice her last words. “You mistake
me a little,” he said. “My reasons for wishing—for thinking it best I
should go away, have nothing to do with my prospects—nothing
whatever. At this moment I have not the faintest notion where I shall