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LYMPHOMATOID GRANULOMATOSIS

&

PAPULOERYTHRODERMA OF OFUJI
PRESENTER: DR. AISHWARYA REDDY
CHAIR PERSON: DR. KALLAPPA HERAKAL
DR. KARJIGI SIDDALINGAPPA
Previous years questions:
• LYMPHOMATOID GRANULOMATOSIS- Clinical features, HPE and
prognosis (RGUHS 2008)

• Explain briefly about -OFUJI PAPULOERYTHRODERMA


( NTRUHS- 2011)
LYMPHOMATOID GRANULOMATOSIS

It is an angiocentric and angiodestructive extranodal EBV-positive B-cell


lymphoma that invariably involves the lungs and may involve the skin.

It is characterized by a perivascular lymphohistiocytic infiltrate with atypical


cells causing lesions of vasculitis.
LG may present at virtually any age,
from late childhood to old age,
but most cases occur in the fourth to sixth decades
with male predominance (2–3:1)
PATHOPHYSIOLOGY

Is an EBV‐driven lymphoproliferative disorder that can be associated with


immunodeficiency states including post‐transplantation and long‐term therapies
such as methotrexate for rheumatoid arthritis.

This lymphoma should be distinguished from extranodal NK/T‐cell lymphoma


(nasal type), which is also EBV+ and characterized by an angiodestructive histology
CLINICAL FEATURES

LG is primarily a pulmonary disease, with lung involvement being present


in >90% of patients at presentation. However, a number of other sites are
commonly affected including the
skin (25–50%)
brain (26%),
kidneys (32%), and
liver (29%).
PRESENTING FEATURES: Patients usually present with chest pain, cough, and
dyspnea in addition to pyrexia, malaise, and weight loss.

The chest radiograph commonly shows bilateral round nodular


opacities or, less commonly, diffuse fluffy infiltration.
CNS involvement is associated with poor prognosis and may manifest
as confusion, ataxia, epilepsy, upper motor neuron signs, cranial nerve
palsies, and peripheral neuropathies.

Dermatological manifestations of LG are varied.


Lesions are usually multiple, and more than one type of lesion may be
present in the same patient.

The most frequent presentation is with dermal and/or subcutaneous


nodules or papules, on the extremities and/or trunk.
Plaques, macular erythema, and maculopapular and folliculitis-like
eruptions have also been described, and rare cases may present as
vesicles, ichthyosis, anhidrosis, alopecia, and necrobiosis lipoidica-like
lesions
Red papules and plaques
over the back
LYG showing extensive, purpuric bruise – like

lesions on the Trunk


Cutaneous lesions may be transient, even when left untreated, and
may precede (10–15% of cases), coincide with, or follow pulmonary
manifestations.

Cutaneous involvement does not appear to influence the outcome of


the disease
PATHOLOGY

Histopathologically, there is a great degree of infiltrate concentrating around


and within the vessels (angiocentric infiltrate), ultimately leading to severe
destruction of the vessels sometimes accompanied by fibrinoid necrosis
(angio-destruction).
The infiltrate is polymorphous and contains both lymphocytes and
histiocytes with pleomorphic or large (immunoblast‐like) tumour cells
and often a prominent reactive T‐cell infiltrate.

Multinucleated cells may be present although well‐formed granuloma


are rare. The presence of large transformed cells is associated with a
worse prognosis.
Grading

The prognosis of LG is, to some extent, a reflection of the histologic grade.


Tumors with a more overt lymphomatous appearance have a more aggres-
sive behavior. Grade is related to the proportion of EBV-positive B cells
relative to the reactive background.
• Grade I lesions are highly polymorphous and show no lymphocytic
nuclear atypia. Blast cells are inconspicuous and often only detected
in immunohistochemically stained sections.
Necrosis is absent or very focal.
EBV+ cells are very sparse (<5 per HPF with in situ hybridization).
• Grade II lesions show lymphocytic cytological atypia, and large
transformed cells are more numerous and
may form small clusters.
Necrosis is common.
EBV+ cells number 5–20 per HPF
Grade III lesions are frankly lymphomatous, although a polymorphous
inflammatory background infiltrate is seen. Large atypical transformed
cells are readily identifiable and may form larger aggregates. EBV+
cells are very conspicuous (>50 per HPF) and may present as confluent
sheets.
When a uniform population of EBV+ blasts is present with no inflammatory
background then a diagnosis of LG is no longer tenable, and the lesion
should be regarded as diffuse large B-cell lymphoma.
Immunophenotype

• The tumour cells are EBV+, express CD20 and are variably CD79aa+.
• CD30 may be expressed but the cells are CD15–.
• The reactive T cells are CD3+ and CD4+.
• Clonal immunoglobulin gene rearrangements can be detected in most
cases and Southern blot analysis usually confirms the presence of
clonal episomal EBV
Disease course and prognosis
Some patients have a fluctuating course with spontane-ous remissions but
eventually progressive disease develops.

Those cases occurring in patients on long‐term methotrexate for conditions


such as rheumatoid arthritis may have a better outcome, with resolution of
disease on withdrawal of the methotrexate
DIFFERENTIAL DIAGNOSIS
• Angiocentric lymphoma
• Chrug Strauss syndrome
• Eruthema nodosum
• Hodgkins lymphoma
• Intravascular lymphoma
• Malignant atrophic papules
• Mycosis fungoides
• Sarcoidosis
• Tuberculosis
• Wegeners Granulomatosis
Management
Although some patients have spontaneous remissions, the
development of high‐grade disease is associated with a median
survival of less than 2 years.

Short‐lived remissions with high‐dose chemotherapy have been described.

There are reports of responses to cyclophosphamide and IFN‐α


PAPULOERYTHRODERMA OF OFUJI
• Papuloerythroderma is as rare dermatoses, occuring primarily in elderly men and
characterised by itchy, monomorphic, red to brown, cobble stone in appearance,
partly confluent, flat topped papules with sparing of skin folds .

• Was first reported by OFUJI et Al in 1984.


EPIDEMIOLOGY
INCIDENCE: More common in Japan.

AGE: Occurs in later lafe, with ages at diagnosis ranging from 57-100yr.
Many cases occur in 8th – 9th decades.

SEX: Male predilection.


M : F – 4.7: 1
ETIOPATHOGENESIS

• Unknown

• Malignacy, Atopic diatheis , Infections , Drug intake are reported to be


possible causes of PEO.
CLINICAL FEATURES
• Presents as awide spread , symmetrically distributed, pruritic eruption of flat
topped, red to brown papules that become confluent.

• There are periodic acute exacerbations, often leading to an erythroderma that


strikingly spares the skin folds – DECK CHAIR SIGN.
Trunk and extremities- typically involved

Spared- face and flexures (ususally)


Mucous membrane , hair, nails –always

Pruritis is consistent ( moderate – severe)


• Palmoplantar keratoderma and Dermatopathic lymphadenopathy are reported
in 20 %of cases.

• Circulating eosinophilia, raised Ig E, lymphopenia – seen in majority of cases.


PATHOLOGY
Histological features – Non specific

EPIDERMIS- often normal in appearance.


But there may be mild spongiosis, mild acanthosis,
and parakeratosis.
DERMIS- a superficial and mid dermal mixed infiltrate composed of
lymphocytes and eosinophils, along with a few plasma cells
and histiocytes, is often seen.
Langerhans cells are increased.

Immunofluorescence: negative.
PROGNOSIS

• Runs a chronic course.

• Persists for many years , some cases may remit.


DIFFERENTIAL DIAGNOSIS
• psoriasis
• cutaneous T-cell lymphoma
• atopic dermatitis
• pityriasis rubra pilaris and
• drug eruptions
• Pityriasis ichenoidess chronica
• lichen planus
• papular eczema
• arthropod bite reactions
TREATMENT
• Systemic corticosteroids are usually effective.
• PUVA alone or in combination with acitretin or etretinate, led to
complete clearance in two-thirds of patients, with an additional
one-quarterr improved.
• Additional reportedly effective treatments include UVB
radiation, cyclosporine, etretinate and azathioprine.
• Topical corticosteroids, however, are usually ineffective as
monotherapy.
SUMMARY

LYMPHOMATOID GRANULOMATOSIS
• angiocentric and angiodestructive lymphoproliferative disease involving
extranodal sites, composed of B cells positive for Epstein Barr virus (EBV)
and admixed with reactive T cells
• Skin is extrapulmonary organ most commonly involved.
• Usually men 40+ years
• Predisposing factor is primary or secondary immunodeficiency states
• Patients may have fever of unknown origin, hemoptysis, history of multiple
skin or other biopsies without diagnosis
• Grading:Relates to the proportion of EBV+ B cells relative to the reactive
background lymphocytes
Grade 1 - infrequent EBV positive cells (< 5/HPF)
Grade 2 - EBV positive large lymphoid cells or immunoblasts (5 - 20/HPF)
Grade 3 - large atypical CD20+ B cells with extensive necrosis and
> 20/HPF EBV positive cells
• Positive stains -CD19, CD20, EBV positive by in situ hybridization,
CD3+ T cells in background
• poor prognosis if constitutional symptoms or multiple organ involvement
• (2/3 die within a year of pulmonary disease and infection)
PAPULOERYTHRODERMA OF OFUJI
• first reported by Ofuji in 1984
• elderly men
• itchy, reddish-brown, partly confluent, flat-topped papules with
sparing of the skin folds (the ‘deck-chair’ sign).
• The etiopathogenesis is not known, but it has been suggested that it can be an
unusual variant of atopic dermatitis, paraneoplastic syndrome (e.g. gastric, lung
and colon cancers), hypersensitivity to drugs, or a pre-lymphomatous condition.

• The association with cutaneous T-cell lymphoma (CTCL) has been increasingly
described.
• It is often associated with lymphadenopathy, peripheral blood
eosinophilia, lymphopenia and elevated immunoglobulin E (IgE)
• Face and flexures – spared
• Systemic corticosteroids are effective.
References:

1) Verma k, Sahni k. Lymphoproliferative disorders.In:Sacchidanand S,


Inandar AC,editors.IADVL Text book of Dermatology.4th Edn.Mumbai:Bhalani
publishing house;2015.p 2154-98

2) Child F, Whittaker S J. Lymphocytic infiltrates.In :Burns T,Breathnach SM,


Cox N,Griffiths C,editors.Rooks text book of Dermatology .9 th Edn.Blackwell
science ;2016.p 140.32-33.
THANK YOU
THANK YOU

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