INFECTIOUS

Disease

Image

Agent

Description/Distribution

Risk Factors/Epidemiology BACTERIA

Pathogenesis

Lab Tests/Dx

Tx

Staph Aureus OR Streptococcus pyogenes

IMPETIGO

Superficial Begins as pustule HONEY-CRUSTED occurs around face esp around nares

Staph Aureus BULLOUS IMPETIGO Staph Aureus, pseudomonas (hot tub folliculitis) FOLLICULITIS

Of all impetigo 30% results in bullous impetigo. Blisters occur at site of infection of Group II phage type 71 Staph aureus superficial infection of hair follicles: erythematous follicular-based papules and pustules beard, post neck, occipital scalp, axillae deeper follicular infection: acute, round, tender, circumscribed, perifollicular staphylococcal abscess that generally ends in central suppuration

5070% of cases are due to S. aureus, with the remainder being due to either S. pyogenes or a combination of these two organisms Group B streptococci are associated with newborn impetigo predisposing factors: minor trauma, pre-existing skin disease, poor hygiene

Streptococci may be early pathogen w/ staphylococci replacing streptococci as the lesion matures s. aureus: exotoxins and coagulase

topical tx: bacitracin or mupirocin be sure to treat around nares to prevent shedding

Exfoliative toxins (ETs): act as serine proteases and can cleave human desmoglein 1, an adhesion molecule in the skin -> causes blister and allows bacteria to spread under the skin -->

Phage typing

topical treatment with clindamycin 1% or erythromycin 2%, coupled with an antibacterial wash or soap

Staph Aureus

pustule enlarge --> tender red nodule --> painful --> RUPTURE --> pain subsides

FURUNCULOSIS

Systemic anti-staphylococcal antibiotics small furuncles: warm compresses large furuncles/carbuncles: incision and drainage MRSA: vancomycin

Staph Aureus CARBUNCULOSIS

coalesced furuncles forming larger draining nodules, with separate heads. back of neck, back, thighs tender, painful, and have fever and malaise nearly always of the shins or dorsal feet begins with a vesicle or vesicopustule, which enlarges and in a few days becomes thickly crusted underlying superficial saucer-shaped ulcer with a raw base and elevated edges remains infection of the skin involving superficial dermal lymphatics local redness, heat, swelling, and a highly characteristic raised, indurated border young children and elderly risk factors: lymphedema, venous status, DM, trauma, alcoholism, obesity B-hemolytic clean w/ soap and water, abx ointment

staphylococcal or streptococcal pyoderma

ECTHYMA

Group A streptococcal ERYSIPELAS

penicillin

s. aureus or strep pyogenes

CELLULITIS

infection extending to subcutis more diffuse w/ ill defined borders and spreads rapidly constitutional sx, regional LAD, sometimes bacteremia

risk factors: lymphedema, venous status, DM, trauma, alcoholism, obesit

untreated --> bullous and necrotic --> abscess/fasciitis

oral Abx: need broad spectrum parenteral therapy for pts w/ extensive disesase, systemic sx, or immunocompromised good hygiene, warm compresses, elevate affected limb

INFECTIOUS

Microaerophilic -hemolytic streptococci, hemolytic staphylococcus, coliforms, enterococci, Pseudomonas, and Bacteroides NECROTIZING FASCIITIS

acute necrotizing infection involving the fascia and subcutaneous tissue Within 2448 h, redness, pain, and edema quickly progress to central patches of dusky blue discoloration, with or without serosanguineous blisters Anesthesia of the involved skin is very characteristic. By the fourth/fifth day, these purple areas become gangrenous w/o tx --> fever, systemic toxicity, organ failure, shock, death

may follow surgery- perforating trauma, or may occur de novo predisposing factors: DM, cirrhosis, IV drug use

CT or MRI: delineate extent of infection biopsy, gram stain, culture to help identify organism

early surgical debridement/fasciotomy, sometimes amputation IV abx: gentamicin and clindamicin supportive care can still have 70% mortality w/ treatment

BACILLARY ANGIOMATOSIS

bartonella

toxin proliferates endothelium cells

treponema palldium SECONDARY SYPHILIS

lesions on palms/soles after systemic infection seeds into skin

VIRUSES acquire virus through inhalation or exposure of mucous membranes may go through several rounds of replication before skin manifestations virus can stay localized if infect specific region exantheums --> virus transmitted via resp route but not contact w/ rash Measles virus maculopapular lesion Koplik spots: gray spots on buccal mucosa prodrome: cough, conjunctivitis, high fever exanthem: hairline, face, neck --> trunks/extremities complications: pneumonia, encephalitis, SSPE prodrome: mild constitutional sx exanthem similar to measles posterior cervical and auricular nodes involved complications: CONGENITAL rubella syndrome, teratogenic Human Parvovirus B19: tiny naked ssDNA slapped cheek syndrome prodrome: mild URI sx exanthem: slapped chekk --> trunk --> central clearing complications: aplastic anemia prodrome: URI sx, abrupt-onset high fever that breaks fine macular rash on trunk --> extremities complications:pneumonia

MEASLES (Rubeola)

RUBELLA (aka German Measles)

ERYTHEMA INFECTIOSUM (5th Disease)

ROSEOLA INFANTUM (6th Disease)

Human Herpes Virus 6 (HHV6): large enveloped dsDNA

INFECTIOUS

Recurrent HSV-1 is the cause 95% or more of the time HSV1 = orofacial HSV2 = genital HERPES SIMPLEX

most frequent clinical manifestation of orolabial herpes is the cold sore or fever blister. typically presents as grouped blisters on an erythematous base HERPES LABIALIS: fever blisters PRIMARY GENITAL INFECTION: erosive dermatitis on external genitalia prodrome: pain, burning, itching

infects mucosal surfaces HSV multiplies in nucleus and surroundes themselves w/ nuclear membrane envelope HSV1: latent in trigeminal ganglion HSV2: latent in sacral ganglion reactivates from psychological or physical stress infectious via contact dormant in sensory ganglia reactivates from immunosuppression, emotional stress/trauma --> dermatomal dermatitis

viral culture: confirms dx acyclovir direct fluorescent Ab: helpful but less specific Tzanck smear: rapid dx but less sensitive

HERPES ZOSTER

Reactivation of Varicella Zoster eruption initially presents as papules and Virus following primary infeciton plaques of erythema in the dermatome. or vaccination Within hours, the plaques develop blisters begins w/ pain/paresthesia in band like pattern post-herpetic neuralgia: continued pain after skin disease resolves

start early on prednisone rest, analgesics, compresses, antiviral therapy disseminated/opthalmict ypes --> IV acyclovir

more than 100 types of HPV

WARTS

benign epidermal neoplasm: hyperplasia + hyperkeratosis elevated, rounded papules with a rough, grayish surface Tiny black dots may be visible, representing thrombosed, dilated capillaries Warts DO NOT have dermatoglyphics (fingerprint folds), as opposed to calluses FLAT WARTS = verruca planar PLANTAR WARTS

Spontaneous resolution (10% stay) Pare the lesion down: CPT code 11055 Topicals: Salicyclic acid Pads (cut to fit). Hold on with duct tape; Aldara. Apply every night (cover with bandaid) Liquid nitrogen: Location dependant 1030sec twice Persistent treatment (every 2-4 weeks): Hemorrhagic Blister is Okay (good sign) Tricks: Forceps and Q-tip

HPV 6, 11, 16, 18 Risk of cervical cancer with Type 16, 18

Incubation can be many months CONDYLOMA ACUMINATUM

MC Viral sexually transmitted disease 30-50% Sexually Active adults have HPV 5% clinical sxs

sexually transmitted direct contact from break in skin --> wart appears 2-9 mo --> wart disrupt adjacent skin --> warts spread infectious via contact

GENITAL WARTS

Liquid Nitrogen: Location dependant usually 10sec Aldara: Apply every night as tolerated 612weeks Sometimes every other night Wash off in the morning Persistent treatment if needed Not completely clear Notify sexual partner(s)

INFECTIOUS

Poxvirus, MCV-1, 2, 3 or 4

MOLLUSCUM CONTAGIOSUM

smooth-surfaced, firm, dome-shaped, pearly papules, averaging 35 mm in diameter may be up to 1.5 cm in diameter central umbilication with central keratotic plug intertriginous sites, axillae, popliteal fossa, groin

3 groups are primarily affected: young Virion is encased in a protective sac children, sexually active adults, and transmission from direct skin or mucous immunosuppressed persons membrane contact virus replicate in cytoplasm --> induce hyperplasia resolve spontaneously but can persist in immunocompromised pts infectious via contact: atopic skin, shaving, bathers, wrestlers, sex

Nothing: 6-8weeks individual; Auto inoculate up to a year Liquid nitrogen with q-tip for 5 seconds Curettage (brutal !) dont use! Leave scar Aldara: AAA qd or qod 3-4weeks

FUNGI Natural habitat is water, soil or decaying vegetation Only a few species are pathogenic for humans. Cell Membrane and Wall Structure They are eukaryotic cells. Cell membrane and wall similar to gram positive bacteria bc theres a cell membrane surrounded by a cell wall Fungal membrane contains ergosterol > different than human cell membranes that have cholesterol Cell wall from inner most layer-out: Chitin -> glucan -> mannoproteins Unlike bacteria it contains no peptidoglycan. KOH Preparation Skin is swabbed with 70% ETOH and allowed to air dry Surface is scraped to remove skin scales or hair that contain the fungus Specimen is treated with 10% potassium hydroxide to destroy tissue elements, specifically keratin (thus KOH is a clearing agent.) Look for branching hyphae or yeast cells.

Unicellular YEAST Multicellular MOLD candida involvement of folds and occurrence of immunocompromised, diabetes, many small erythematous desquamating elderly, pts on abx satellite or daughter lesions scattered along the edges of the larger macules CANDIDAL INTERTRIGO: intertriginous areas often affected bc inc warmth, moisture, and maceration, permitting organism to thrive --> becomes reddened plaques ORAL THRUSH = oropharyngeal candidiasis: easily scrapable nonadherent plaques, dysphagia PARONYCHIA: infection of nail w/ tender, edematous, erythematous nail folds w/ purulent discharge CANDIDAL VULVOVAGINITIS: acute inflammation of the perineum characterized by itchy, reddish, scaly vaginal mucosa; and creamy discharge. BALANITIS: shiny reddish plaques on glans penis

Reproduce by budding. Some also produce pseudohyphae. Produce hyphae usually on skin flora --> altered host env leads to proliferation Solid media: grow as smooth colonies and look very much like bacteria KOH prep: budding yeast, pseudohyphae

fluconazole 1x/week, stays in skin/hair/nails for 6-7 days no talcum --> irritating CANDIDA INTERTRIGO, BALANITIS: topical antifungals (azoles) THRUSH: nystatin or clotrimazole PARONYCHIA: topical antifungal 2-3 mo, PO anti-staph abx VULVOVAGINITIS: fluconazole

CANDIDIASIS

SUPERFICIAL MYCOSES

INFECTIOUS

Malassezia furfur PITYRIASIS VERSICOLA

hypo- or hyperpigmented coalescing scaly macules on the trunk and upper arms

Heat and humidity systemic corticosteroid use, cushing's, immunosuppression, malnutrition

Dicarboxylic acids inhibit melanin Sold media: produce production by inhibiting tyrosinase --> filamentous colonies hypopigmentation griseofulvin and lamisil do not work --> more for dermatophytes

ALL TINEA INFECTIONS Hortae Werneckii

Survive on dead keratin Classified by body region

Topicals: Powders Domeboro solution 20min TID Selenium sulfide for hair Azoles creams: Some have antibiotic capabilities (spectazole) KOH from scale at border Lamisil or nafitine bid x 2-4weeks (fungicidal) Get a culture

dimorphic fungus

TINEA NIGRA

Strands of mycelium and numerous spores called spaghetti and meatballs when viewed microscopically (KOH prep) Wood's light exam acentuates pigment changes

Oral Location (scalp and groin) and size dependant Griseofulvin 20-25mg/kg/d divided BID and Lamisil 2-6weeks

Piedra hortae BLACK PIEDRA Trichosporon beigelii WHITE PIEDRA

CUTANEOUS MYCOSES Trichophyton rubrum 1 T. mentagrophytes Epidermophyton floccosum Affected skin is usually pruritic, with scaling Adolescents and young males plaques on the soles, extending to the most common fungal infection in lateral aspects of the feet and interdigital north america and europe spaces often with maceration.

topical: terbinafine, clotrimazole, econazole

TINEA PEDIS aka Athlete's Foot

T. rubrum T. mentagrophytes TINEA UNGUIUM

thickened yellow nails and hyperkeratotic subungual debris

TINEA CRURIS

T. rurum E. floccosum

jock itch: in groin, upper/inner thighs but common in men spares the groin scaling annular plaques Ringworm infection face, hands, body infection of palms of hand infection of : scaly erythematous skin w/ hair less common children Especially in children

TINEA CORPORIS TINEA MANUUM

Caused by any dermatophyte T. rubrum ECTOthrix- caused by Microsporium audouinii, Microsporium canis and some trichophyton. ENDOthrix- caused by Trichophyton tonsurans

TINEA CAPITIS

hyphal elements and arthrospores SURROUND the hair shaft Hair breaks a few millimeters ABOVE the scalp Arthrospores INSIDE the shaft itself Hair breaks AT the scalp

TINEA BARBAE

T. mentagrophythes T. verrucosum

infection of beard hair

AUTOIMMUNE/INFLAMMATORY

Disease

Image

Description/Distribution Wheals, many diff shapes and sizes can migrate

Risk

Pathogenesis Type I hypersensitivity chronic types: still can rarely find a cause

Lab Tests/Dx

Tx supportive

URTICARIA

eosinophils

antihistamines: H1 blockers, H2 blockers, doxepin steroids: long taper avoid irritants/allergen barrier cream: zinc oxide antihistamines topical steroids patch testing

CONTACT DERMATITIS

pruritic, erythematous oozing rash

contact to poison ivy, nickel, rubber, preservatives

Type IV hypersensitivty to IRRITANTS (larger than Ag/allergens)

red poorly defined plaques w/ scale and crust on cheeks infants/toddlers: cheeks, forehead, EXTENSORS older children/adolescents: flexural areas of neck, elbows, wrists, ankles adults: FLEXURAL areas of wrists, ankles, feet, face dry skin/xerosis diaper area spared Dennie-Morgan lines: double folds and lines under eyes from chronic edema Horizontal nasal crease: from constantly scratching nose

associated w/ asthma chronic pruritic inflammatory and allergic rhinitis skin disease most common in developed countries genetics: fillagrin mutation --> cause transepidermal water loss impaired immune response environment/stress COMPLICATIONS: Lichenification: thick callous skin from chronic trauma Neurodermatitis: itch-scratch cycle, becomes subconscious from scratching

MOISTURIZE

Avoid perfumes, soaps, hot water antihistamines Topical steroids: OINTMENTS stronger, avoid use in intertriginous areas

ATOPIC DERMATITIS

Pityriasis alba: small hypopigmented ill-defined patches on face from inhibition of melanin due to thicker skin

Scars Infections: impetigo, warts, molloscum Eczema herpeticum: HSV can use atopic dermatitis to invade and spread into skin comedome (pore) --> oil and keratin builds up --> ruptures hair follicle --> bacteria accumluates --> inflammatory response --> PUSTULES Abx: for skin infections but use sparingly

Comedomes: topical retinoids, accutane

ACNE 4 factors: follicular hyperkertinization, excess sebum, bacteria, inflammation Bacteria: Abx, benzoyl peroxide Inflammation: Abx, benzoyl peroxide, hormonal therapy, accutane Sebum production: hormonal therapy, accutane erythematous targetoid lesions in palms or mucous membranes, B/L and symmetric immune complex formation -> deposit in cutaneous microvasculature work up: HSV titers, skin biopsy early histologic findings: vacuolar drugs: sulfonamides, interface dermatitis, perivascular anticonvulsants lymphocytic infiltrate late histologic findings: subepidermal bullae, full thickness necrosis

PO prednisone

ERYTHEMA MULTIFORME

20-40 yo

acyclovir 4x/day

infection: HSV, mycoplasma

idiopathic

widespread blisters and purpuric macules. mucosa involvement and < 10% epidermal detachment STEVEN JOHNSON SYNDROME

from meds: abx, antiepileptics, NSAIDs

death rate: 1-5%

initial sx: fever, stinging eyes, swallowing severe, denudation (>30% epidermal detachment), also from meds TOXIC EPIDERMAL NECROLYSIS

true derm emergency --> high morbidity and poor prognosis

death rate: 34-40%

transfer to ICU/burn unit

AUTOIMMUNE/INFLAMMATORY

TOXIC EPIDERMAL NECROLYSIS

Nikolsky's sign: lateral pressure induce separation of epidermis from dermis

great loss of water/fluids --> need to go to burn unit increased risk for infection --> die from SEPSIS

steroids controversial: can't fight off infection

ERYTHEMA NODOSUM

fat disorder on extensor surfaces of extremities, more common on ant shins prodrome: fever, joint pain, malaise well demarcated thick erythematous plaques w/ silver scale on extensor surfaces PLAQUE: silver scale and sharp demarcations, symmetric. Most common type INVERSE/FLEXURAL: bright red plaques in skin folds with no scales (often misdiagnosed as candidiasis) GUTTATE: Rain-drop sized salmonpink scaly papules on trunk or extremities. Usually after strep infection PUSTULAR: Sterile pustules in cornified layers, on palms and soles. Often from withdrawal of corticosteroids --> AVOID ORAL STEROIDS FOR PSORIASIS

hypersensitivity rxn to: URI, infection, IBD, malignancies, sulfonamides, Ocs rash occurs for 1-3 wks then resolves T cell mediated: releaseTNF- and interleukin cytokines clinical body starts to produce skin at histology : acanthosis, elongated faster rate rete ridges, hyperkeratosis and parakeratosis

IVIG: best txNSAIDs, but expensive supportive: supersaturated solution of potassium iodide in orange juice, PO prednisone

Abx for URI Topical steroids (NOT ORAL): potent except for intertriginous areas

Calcipotrene: Vit D derivative

OTHER FINDINGS: Psoriatic Arthritis: Sausage digits, tenosynovitis, affects DIP joints or sacroiliitis

phototherapy: UVB, excimer laser, PUVA

PSORIASIS

systemic (for psoriatic arthrits): MTX, cyclosporine, acitretin

Nail changes: Pitting, oil spots, onychodystrophy Auspitz sign: pinpoint bleeding after picking off scale, from vessels in dermal papillae Scalp involvement Geographic tongue: Plaque psoriasis on tongue, may have bad breath high risk for CVD histology: hyperkeratosis, acanthosis, saw-tooth elongation of rete ridges, lymphocytic infiltrate

Biologics: TNF-a inhibitor, IL 12/23 blocker

ERYTHRODERMIC: Bright red lesions involving full body w/ fevers/chills/malaise. Often from uncontrolled or untreated psoriasis --> need hospitalization

intralesional kenalog injections

LICHEN PLANUS

purple, pruritic polygonal papules widespread papulosquamous eruption

LICHEN SIMPLEX CHRONICUS

from repetitive trauma/scratching

histology: acanthosis, hyperkaratosis, hypergranulosis, elongated rete ridges, fibrosis of papillary dermis

Flaccid blisters and erosions w/ frequent mucous involvement Nikolsky sign: intact epidermis shearing away from dermis --> leave moist surface behind PEMPHIGUS VULGARIS Asboe-Hansen sign: put pressure on intact bullae fluid spreads under adjacent skin and makes blister bigger 40-60 yo, M = F

IgG against Dsg 3 --> lose normal adhesion

histology:

suprabasilar acantholysis intraepidermal bullae blisters/separates within epidermal layer tombstone row of basal layer keratinocytes immunofluorescence: chicken wire/fish net pattern

PEMPHIGUS FOLIACEUS

Flaccid bullae and localized or generalized exfolication, rapidly denudes face, scalp, upper trunk intact bullae, erythematous papules, urticarial plaques involving skin and mucosa can be in groin, axillae, trunk, thighs

middle aged and elderly

IgG against Dsg 1

histology: Subcorneal acantholysis

BULLOUS PEMPHIGOID

60-80 yo, M=F

histology: Subepidermal bulla with IgG against basement eosinophils or neutrophils membrane /hemidesmosomes (BP230, BP180) immunofluorescence: linear

AUTOIMMUNE/INFLAMMATORY

DERMATITIS HERPETIFORMIS

Intensely pruritic papulovesicular eruption symmetrically on elbows, knees, buttocks

20-60 yo, males

Associated w/ glutensensitive enteropathy histology: neutrophils in HLA-B8, HLA-DR3, HLA-DQw2 subepidermal space Type III hypersensitivity: granular IgA in papillary dermal tips hair specific autoimmune disease Lymphocytic infiltrates sometimes around or within hair bulb of anagen follicles can be stress induced no tx: 90% clear within one year Minoxidil (rogaine) Topical steroids: if autoimmune, < 10 yo intralesional steroid injections foreign body inflammatory rxn to dark coarse curly hair shaving --> hair curls and dives back into skin --> brings in bacteria stop shaving

non-scarring alopecia/balding in round defined area ALOPECIA AREATA asymptomatic: no scale, erythema, orLAD regrown hair can be white

PSEUDOFOLLICULITIS BARBAE

transfollicular or extrafollicular penetration of foreign bodies bumps proliferate with more shaving

use single blade/electric razor retin-A: decrease hyperkeratosis topical corticosteroids oral or topical abx: for pustules/abscesses and reduce skin bacteria laser hair removal: painful and only works short-term pustular --> short-course of abx

ACNE KELOIDALIS NUCHAE

chronic scarring folliculitis

chronic irritation: close haircuts, rubbing of head gear

hyperkeratosis --> retin-A gel, high potency topical steroids scar --> intralesional steroids TAC excision in stages

SYSTEMIC/CONNECTIVE TISSUE DISEASES

Disease

Image

Description/Distribution

Pathogenesis

Lab Tests/Dx

Tx

CONNECTIVE TISSUE DISEASES systemic disease involving skin (malar rash, discoid rash), oral ulcers, heart, kidneys, joints, neuro, blood

type III hypersensitivity Anti Ro/La (ssDNA) sun avoidance

cell rupture releases "garbage" DNA --> macrophages can't clear suspect if have frequent miscarriages completely --> Ab made against DNA/histone proteins --> complexes deposit throughout body

SLE

ACUTE CUTANEOUS LE form: looks like SLE, photodistributed butterfly rash. No internal involvement

ANA: main screening test -> 1:160 = positive. but have false positives (infection, elderly, pregnant) need 4 of 11 criteria: malar rash, discoid rash, photosensitivity, oral ulcers, arthritis, serositis, renal disorder, neuro disorder, hematologic disorder, immunologic disorder, antinuclear Ab work up: blood work, biopsy

topical steroids

plaquenil: dec immune system and reduce sun sensitivity but need optho exam before starting

SUBACUTE LE: photodistributed annular plaques, joint and vasculitis involvement CHRONIC/DISCOID LE: photosensitivity atrophic plaques on scalp --> scarring alopecia. Rarely ANA positive NEONATAL LUPUS: mother pass Ab to kids --> kids get anti-Ro and heart block DRUG INDUCED LE: from hydralazine, procainamide, isoniazid proximal muscle weakness with cutaneous manifestation adult variant: associated w/ internal malignancy --> SCREEN for cancers child variant: calcinosis of skin DERMATOMYOSITIS heliotrope rash Gottron's papules: raised fleshy colored lesions periungual erythema and telangiectasia: capillary loops in proximal nail bed thrombose/break shawl sign: violaceous scaling patches around shoulder diffuse, hard immobile skin chronic fibrosing systemic disease --> fibroblasts activated to make more collagen

IL kenalog

immunosuppressants: Imuran, MTX

Ab tests: ANA, Jo-A, SS-A (ro), t-RNA synthetase muscle biopsy = gold standard

topical steroids LOTS of oral prednisone add steroid sparing agent w/in 2 months: MTX, imuran, celicept avoid sun plaquenil

MTX

SCLERODERMA

SYSTEMIC SCLEROSIS: diffuse and CREST syndrome (calcinosis, raynaud's, esophageal involvement, sclerodactyly, telangiectasia)

systemic fibrosis: have resp (interstitial fibrosis, pulmonary HTN), CV, GI, kidney issues. Most die from renal HTN diffuse: anti-scl70

Raynaud's --> calcium channel blocker

LOCALIZED SCLEROSIS: morphea --> just involves skin. atrophic plaques (skin steps off) w/ telangiectasia. Middle aged females

limited: anti-centromere

penicillamine ACE: renal protection morphea tx: IL Ken at leading age, topical CS, plaquenil

VASCULITIS non-blanchable spots on trunks/legs that are raised --> palpable purpura GIANT CELL/TEMPORAL ARTERITIS U/L headache near temples pulseless disease: fever arthritis, weak pulses in extremities need to dx quickly or else may turn BLIND

punch biopsy

PO steroids and refer to derm

biopsy temporal artery

HIGH DOSES of PO steroids

TAKAYASU ARTERITIS

SYSTEMIC/CONNECTIVE TISSUE DISEASES

granulomatous inflammation C-ANCA (anti-pr3) positive of resp tract necrotizing vasculitis of smallCXR: migratory infiltrates med sized vessels necrotizing glomerulonephritis UA: RBC/casts triggers: vaccine, LTinhibitors, cocaine, azithromycin, rapid stop of steroids

steroids + cyclophosphamide second line: IVIG, cellcept, rituximab, azathioprine fatal if not tx aggressively

friable erythematous gingiva: strawberry mouth

P-ANCA (anti-MPO) positive

steroids cytoxan: if have cardiac disease

CHURG-STRAUSS SYNDROME

lung and heart involvement

associated allergic rhinitis, nasal polyps, asthma

eosinophil-rich and granulomatous inflammation CBC: peripheral eosinophilia of resp tract necrotizing vasculitis of smallmed sized vessels heart disease = most common cause of death necrotizing vasculitis of small vessels necrotizing glomerulonephritis P-ANCA positive

SQ nodules: may ulcerate MICROSCOPIC POLYANGIITIS

steroids 1 mg/kg/day to start cyclophosphamide or azathioprine: for severe renal or lung disease IVIG

lung and kidney involvement

prodrome: fever, arthalgia, myalgia, weight loss splinter hemorrhages, ulcers, necrotizing livedo

pulmonary capillaritis type III hypersensitivity: IgA dominant immune deposits in small vessels

affects kids HENOCH-SCHONLEIN PURPURA skin, gut, kidney involvement preceded by URI strep or other infectious triggers

need serial UA to track kidney function IF: IgA, C3 and fibrin deposition in affected vessel walls

self-limited Dapsone, Colchicine: dec duration of cutaneous lesions systemic corticsteroids: for arthrlagias and abd pain

usually clincial dx palpable purpura in lower extremities refer to nephrologist LEUKOCYTOCLASTIC VASCULITIS headache, joint pain (LE large joints), hematuria, rash, abdominal pain waste basket dx cryoglobulin immune deposits in skin and glomeruli --> precipitate out w/ cold and occlude blood vessels congenital capillary malformation: deficiency of SNS innervation of vessels color gets dark w/ crying, fever, overheating in < 1% of newborns can evolve to raised, thickened plaque

from drugs, URI

CRYOGLOBULINEMIC VASCULITIS

no signs of systemic disease or glomerulonephritis

NEVUS FLAMMEUS

port wine stain in head and neck: dermatomal, unilateral, variable blanching

CUTANEOUS MANIFESTATIONS OF INTERNAL DISEASE

SYSTEMIC/CONNECTIVE TISSUE DISEASES

ACANTHOSIS NIGRICANS

velvety hyperpigmented plaque

Nonspecific reaction pattern that may accompany obesity; diabetes; excess corticosteroids; pineal tumors; other endocrine disorders

determine type of xanthoma build-up of lipids in tissue due to dyslipidemia measure fasting cholesterol, TAGs, HDL, VLDL, LDL primary hyperlipoproteinemia = dx of exclusion congenital lesions of skin, CNS, bone, autosomal dominant endocrine glands congenital disease > 2 or more features = NF1 dx excise cutaneous tumors MRI: dx, management, and follow pt closely to detect screening of family malignant degeneration of members neurofibromas

XANTHOMAS

lipid abnormalities

> 6 caf au lait spots

NF1 gene

axillary freckling: earliest sign NEUROFIBROMATOSIS 1 genetic counseling cutaneous neurofibromas NF1 + juvenille xanthogranuloma = HUGE risk of juvenille CML

Lisch nodules: iris hamartomas

bone lesion: sphenoid wing dysplasia or congenital bowing of long bones

bilateral acoustic neuroma

autosomal dominant

NEUROFIBROMATOSIS 2

1-2 caf au lait spots SCH gene: swannomin/merlin proteins defective

neurofibromas: less common than NF1 no lisch nodules or mental retardation/learning disabilities caf au lait, low set ear, webbed neck NOONAN SYNDROME short stature, pectus excavata pulmonic stenosis, cryptorchidism, hypogonadism short, facies, low set ears
CARDIOFACIOCUTANEOU S SYNDROME

autosomal dominant: PTPN11 mutation

BRAF, MEK1, MEK2 mutations caf au lait, cardiac defects

MCCUNE ALBRIGHT SYNDROME

large segmental caf au lait bone lesions: polyostotic fibrous dysplasia

sporadic: GNAS1 mutation

precocious puberty, hyperthyroidism

LEOPARD SYNDROME

caf noir spots: more black than creamy PTPN11 mutation

SYSTEMIC/CONNECTIVE TISSUE DISEASES

LEOPARD SYNDROME EKG conduction defects, deafness, hypospadias/cryptorchidsm Multiple hamartomas (benign tumors) of the skin, central nervous system, kidneys, heart, retina, and other organs

PTPN11 mutation

autosomal dominant MRI brain genetic counseling

epilepsy + angiofibromas + mental retardation adenoma sebaceum shagreen patch: usually in lower trunks

TSC1, TSC2 gene defect

surgical excision of facial lesion if cosmetic concern

inc chance of malignancy

TUBEROUS SCLEROSIS

white ash leaf macules: hypopigmented ellipitic macule. Earliest finding

periungual fibromas: tumors around nails that look like warts brain lesions: glioneuronal hamartoma --> cause seizures, cognitive defects, autism, behavioral problems heart: cardiac rhabdomyoma renal: angiomyolipoma, renal cell carcinoma opthalmic: retinal hamartoma pulmonary: lymphangioleiomyomatosis --> SOB and PTX multiple hamartoma syndrome COWDEN DISEASE multiple facial trichilemmomas: looks like wart oral mucosal papillomatosis < 10% have caf au lait spots sebaceous gland tumor + > 1 internal malignancy most common associated neoplasm = colorectal cancer FAP + extraintestional manifestations GARDNER SYNDROME intestinal polyposis, epidermal cysts, multiple osteomas, desmoid tumors epidermal cysts on weird locations (legs) fibrofolliculomas, achrochordons, trichodiscomas BIRT-HOGG-DUBE SYNDROME autosomal dominant: PTEN mutation high incidence of malignant tumors of breast and/or thyroid gland

MUIR-TORRE SYNDROME

autosomal dominant

can get spontaneous PTX and renal cancers

autosomal dominant

BENIGN LESIONS

Disease

Image

Description/Distribution

Pathogenesis

Lab Tests/Dx histology: MATURE melanocytes descending into dermis

Tx

benign neoplasm of melanocytes

JUNCTIONAL NEVUS (A): flat, at epidermal/dermal junction MELANOCYTIC NEVUS INTRADERMAL NEVIS (C): indurated, in dermis w/ no connection to epidermis COMPOUND NEVUS (B): still connected to epidermis but growing down into dermis brown pigmented lesion w/ hair present at birth --> monitor carefully b/c can become melanoma if large CONGENITAL NEVUS BECKER'S NEVUS: common on men in lateral upper chest. Lots of hair associated serial excisions for prophylaxis before turns into melanoma

HALO NEVI

immune rxn against mole from body

can get vitiligo

benign juvenille melanoma: most common in children SPITZ NEVUS hairless, red/brown dome-shaped papule slightly elevated, round, regular nevus pigment in dermis --> reflects blue light remove

BLUE NEVUS

LABIAL MELANOTIC MACULE

faint black spot on lower lip/mucosa

DYSPLASTIC NEVUS

irregular pigmented macule w/ irregular border, smaller than melanoma

marker for inc risk for developing melanoma

histology: atypical melanocytes w/ bridging of remove mod and rete ridges severe-graded atypia

cutaneous hamartoma: contains surface epidermis and adnexal structures (sebaceous gland) linear, unilateral, wart-like, whorled follow Blashkos lines: where melanocytes travel in utero

EPIDERMAL NEVUS

present at birth

cryo, surgery, laser

ILVEN (inflammatory linear verrucous epidermal nevus): intensely erythematous, pruritic, inflammatory

usually scalp

present at birth

NEVUS SEBACEOUS

BENIGN LESIONS

tumors can arise on top: need prophylactic surgical excision NEVUS SEBACEOUS linear --> cobblestoned common benign neoplasm = trichoblastoma common malignancy = BCC BCC MIMICS multiple flesh-colored small papules around eyes, from eccrine glands around eyes

SYRINGOMA

histology: comma-shaped groups of epithelial cells

TRICHOEPITHELIOMA

small flesh-colored papules on face turban tumor = autosomal dominant

histology: pseudo-horn cysts

CYLINDROMA

flesh-colored to reddish nodule on head, neck, scalp

histology: jigsaw-puzzle pattern

PILOMATRICOMA

firm nodule in a child w/ erythematous surface

histology: basaloid cell nest, ghost cells (no nucleus), giant cells, bone cells and calcifications

raised discolored plaques on extremities or face w/ "stuck on" appearance benign squamous proliferation

genetic predisposition: rare if < 30 sign of skin maturity --> usually in elderly

histology: horn cysts well-circumscribed, crumbles when picked

shave removal curettage liquid nitrogen: be careful on dark skin -> can leave hypopigmentation

doesn't occur on lips, palms, soles

raised and brown = okay, flat and black = problem

IRRRITATED SK: often confused w/ BCC due to inflamed/swollen look

SEBORRHEIC KERATOSIS

LESER-TRELAT SIGN: eruptive SK as sign of internal malignancy (usually gastric cancer)

STUCCO KERATOSES: papular warty lesions on lower legs, sign of dry skin

DERMATOSIS PAPULOSA NIGRA: multiple brown-black papules on african-americans. "morgan freeman" disease

silicone gel sheeting: doesn't work tender, itches, grows fast KELOIDS scar that proliferates beyond margin of injury

BENIGN LESIONS

tender, itches, grows fast KELOIDS scar that proliferates beyond margin of injury

more common in blacks invagination of epidermis w/ black punctum, very common EPIDERMAL INCLUSION CYSTS pore from old hair follicle invaginates into skin and grows keratin --> bacteria makes abscess around it spontaneously ruptures -> need to destroy cyst wall to prevent recurrence multiple EICs: suspect Gardner's syndorme

intralesional steroid injections: large needle to inhibit collagen synthesis cryotherapy, compression, irradiation

drain fluctant and inflamed cysts

commonly on scrotum PILLAR CYST: form of EIC without punctum, on scalp. Filled w/ hard debris

small punch biopsy over black punctum

ACROCHORDON

skin tags: tiny, brown/skin colored and attached by short narrow stalk

from chronic irritation, rubbing, genetics, obesity

snip excision w/ iris scissors large tag --> shave removal cryotherapy: spray or forceps

CUTANEOUS HORN

hard conical projection made of keratin

a wart, actinic keratosis, or SCC

surgical excision cryo doesn't work dimple sign: lesion retract beneath skin when you compress and elevate w/ thumb and index finger

asymptomatic to slightly itchy lesions more in females DERMATOFIBROMA pinkish papule w/ darker hyperpigmented ring, usually on leg fibrous rxn to trauma, viral infection, insect bite multiple DFs seen in SLE inflamed cartilage CHRONDRODERMATITI S NODULARIS HELICIS pain out of proportion if suspect AK women: antihelix men: helix

excision: ellipitical or punch, depending on size

LN2 cut out cartilage donut pillow: relieve

MALIGNANT LESIONS

Disease MELANOMA

Image

Subtypes

Description/Distribution malignant neoplasm of melanocytes

Risk Factors/Epidemiology Pathogenesis Growth Phases Radial growth: superficial and laterally Vertical growth: deeper into dermis Pathogenesis familial melanoma: CDKN2A mutation --> Rb and p53 inactive MC1-R gene: increases risk Better Prognosis factors young age female extremities skin metastasis better than visceral

Lab Tests/Dx ABCDE asymmetry, border irregularity, color, diameter Color: dark, brown, or pale Metabolic Panel +S100 HMB45 Melan A Histology Atypical cells in epidermis Lack of maturation with descent Cytologic atypia & nuclear pleomorphism Nucleolar variability: often large & irregular Mitoses: deep dermal, often atypical Increased apoptosis

Tx biopsy depth > 1 mm: need sentinel LN biopsy LN pos = metastatic --> dissect sentinel and surrounding LN LN neg: remove primary tumor EXCISION in situ: 0.5 cm margin < 1 mm: 1 cm margin down to fascia > 2 mm: 2 cm margin down to fascia Adjuvant Therapy IFN-a: inc survival but not well tolerated Metastatic disease CT: chest, abd, pelvis MRI brain radiation

personal history of atypical moles, fam hx congenital nevus nonmelanoma skin cancer immunosuppression sunburn, PUVA, chronic tanning, white skinned UVA >> UVB

Melanoma in Situ

histology: atypical melanocytes but basement membrane still intact

Breslow Depth obtain skin biopsy for dx and staging predicts survival and prognosis measures top of granular layer to deepest point of invasion < 6mm: benign > 0.76mm DEEP -> regional lymph node involvement so inc

Superficial Spread

lower leg in female MC type of MM in the begins as flat lesion back/trunk in males white-skinned population can turn black, blue, red, In early states it may be small, 70% of cases white then growth becomes irregular horizontal superficial spread w/o much induration Trunk is a common site M>F Usually with a POOR prognosis Black/brown nodule Ulceration and bleeding are common rapid growth vertical growth phase

Nodular

MALIGNANT LESIONS

Lentigo Maligna Melanoma

Mainly on face in elderly pt grows on epidermal-dermal junction

Elderly pts

May be many years before an invasive nodule develops grows on sun damaged skin

Acral Lentiginous Melanoma

Usually comprises a FLAT lentiginous area w. INVASIVE nodular component POOR prognosis b/c usually identified too late --> on palms or soles

Common in black people KIT gene: overexpressed. and Asian possible gene association In white-skinned population accounting for 10% of MM

hutchinson's sign: pigment spreads under nail plate to proximal and lateral nail folds

Subungal Melanoma

Often diagnosed late Confusion with benign subungal nevus, infections or trauma

Rare

Amelanocytic Melanoma fleshy pink, indurated lesion Diagnosis is often missed clinically so do bx often mistaken for BCC or verruca

Lack of pigmentation due to the rapid growth of tumor and differentiation of the malignant melanocytes

Desmoplastic Melanoma

Usually found on head and neck region Usually amelanotic lesions High chance for recurrence

Positive S-100 protein but are often negative to tyrosinase, Melan-A.

Conjunctival Melanoma Markedly pigmented tumor in eye

Metastatic Melanoma Satellitosis near the primary melanoma Sometimes distant metastases that appear as papules or plaques, not pigmented.

MALIGNANT LESIONS

Malignant Melanoma Invasive and may show lymph node invasion by tumor cells (pics on right)

Rate of Metastasis pTis (in situ): 5 mm margin pT1 (<1.0 mm): 1 cm margin pT2 (1.02.0 mm): 12 cm margin pT3 (2.04.0 mm): 12 cm margin pT4 melanoma (>4.0 mm): 2 cm margin MC type of skin CA fair skin, blue eyes, fair hair Inability to tan Exposure to UV radiation from sunlight or artificial tanning lamps UV exposure patternintermittent, childhood sun exposure immunosuppression PTCH mutation: unchecked cell proliferation photocarcinogenesis: DNA damaged by UVB, ROS induces DNA damage, cell-mediated immunity suppressed, p53 mutates p53 mutation: can't trigger apoptosis indolent, low metastatic potential New drug target PATCH called Vismodegib ELECTRODESICCATION AND CURETTAGE (ED&C): great for small low risk BCC Excision: large low risk (5 mm) or any high risk (10 mm) Nests or lobules of MOHS micrographic surgery: hyperchromatic but uniform microscopically controlled basaloid cells with PERIPHERAL technique with real-time PALISADING surrounded by frozen tissue secitons to loose stroma insure margin control; for high CLEFT-LIKE retraction spaces risk, large low risk, face, scalp, May appear pigmented due and neck, recurence to dermal melanophages Radiation- adjunctive therapy if can't do surgery, or if have perineural invasion Aldara- low cure rate When washing face, crust comes off and lesion starts bleeding Almost always epidermal attachment MOHS excision w/ 4 mm margins EDC XRT imiquimod topical 5-FU photodynamic therapy, cryosurgery combination therapy: EDC + imiquimod

Derived from basal cell layer of epidermis Head & neck of elderly patients Slow, progressive growth Locally destructive, recurs, rarely metastasizes translucent, "pearly" nodule w/ telangiectasia high risk: micronodular, infiltrative, morpheaform, pigmented types

Nodular BCC

low risk

Superficial BCC

Erythematous plaques w/ +/papules, scale, telangiectasia

low risk

BASAL CELL CARCINOMA

Morpheaform BCC

scar-like or white plaque w/ slight translucence. Looks like scar on nose well-marginated, red or fleshcoloured nodule with cystic centers. POPPING DOES NOT RESOLVE IT --> not just a simple cyst smooth pink plaque on lower back. Very rare

high risk

Cystic BCC

Fibroepithelioma of Pinkus

histology: thin anastomosing strands and cords of tumor cells extending into dermis from epidermis surrounded by fibrous stroma

MALIGNANT LESIONS

Gorlin Syndrome aka Basal Cell Nevus Syndrome

Hundreds of Basal Cells that look like moles Also get calcification of falx cerebri, jaw cysts, bifid ribs

Auto dom PATCH gene mutation Kids

SQUAMOUS CELL CARCINOMA

Excellent prognosis erythematous, keratotic papule or nodule Metastases uncommon if tumor < 1.5 cm deep 5% metastasize if 2 cm or more and definite dermal invasion metastasizes usually to lung

Usually men 2nd MC skin cancer Very rare in blacks UVB > UVA exposure transplant pts: need frequent derm follow-up other exposures: chronic ulceration/inflammation, scarring, arsenic from wells, smoking HPV 16/18

risk factors for metastasis: ear/lip, impaired host, > 2 cm, depth > 4 mm, perineural invasion, poor differentiation

Irregular border Indurated with white/yellowish scale Can see hyperkeratosis/parakeratosis Can see cells along the basal layer are mildly irregular, some are large w/ hyperchromatic nuclei Stay in bottom 1/3 of epidermis The dermis is bluish keratin pearls

Surgical excision with adequate margins ELECTRODESICCATION AND CURETTAGE (ED&C) Excision: high risk (10 mm), low risk (4 mm) MOHS Radiation-adjunctive therapy Aldara-low cure rate if palpable LN: fine needle/surgical biopsy

Keratoacanthoma

Actinic Keratosis

rapidly growing ulcerative keratotic nodules that looks like crater GRYZBOWSKI SYNDROME: multiple nonregressing, generalized eruptive FERGUSON SMITH SYNDROME: familial, spontaneously regressing KERATOACANTHOMA CENTRIFUGUM MARGINATUM: solitary, expanding erythematous plaque w/ central regression. looks like discoid Very Common, precursor to SCC that's confined to epidermis mild erythema w/ imperceptible scale --> becomes more papular and obvious later cutaneous horns can overlie AK

elderly ~ 64 yo more prevalent in MuirTorre syndrome pts

can spontaneously regress

surgical excision: if regress on own will heal w/ scarring MTX injection, topical 5-FU, imiquimod radiation

Caused by long term UV exposure sun-damaged skin on balding scalp aka dermal solar elastosis Worse in transplant patients

solar elastosis: greyish areas in dermis dysplastic cells in lower epidermis, hyperkeratosis need inspection and palpation to detect, may be imperceptible

LN2 (cryotherapy) topical chemo like 5-FU or Aldara sunscreen photodynamic therapy

MALIGNANT LESIONS

Actinic Cheilitis

diffuse AK/change of lip loss of vermilion border squamitization of mucosal tip leukoplakia

SCC can arise from actinically damaged tip --> can metastasize in mouth and spread to throat

Marjolins ulcer

SCC coming from area of scar/chronic inflammation ulcer that doesn't heal

incisional biopsy

Bowen's Disease aka SCC in Situ/ Erythrodysplasia of Queyrat

Full thickness epidermal atypia but not into the dermis larger size, induration, crusting, refractory to LN2 --> BOWEN'S

can arise in non-sun exposed NOT invasive bc BM is intact sites Dysplastic throughout risk of progression to SCC epidermis

LN2, topical chemo like 5-FU or Aldara, or surgery. If on genitalia, do MOHS

Leukoplakia

MC premalignant lesion of the oral mucosa, treat like SC-Cis

ddx = thrush, but this lesion NON-SCRAPABLE

KARPOSI'S SARCOMA

Spindle-cell tumor derived from endothelium Caused by Human Herpesvirus 8 (HHV8) aka KSHV bluish-red or purple bumps on the skin (from vascular lesions from epithelium)

Epidemic, AIDS-related Immunocompromised Classic or sporadic Endemic (African)

irregularly diliated anastomosing vascular channels

HPV associated

large well-differentiated nests of eosinophilic keratinocyts w/ "pushing border"

Oral florid papillomatosis VERRUCOUS CARCINOMA

mouth

Giant condylomata of on genitalia Buschke-Lwenstein

SurgeryDO NOT Radiatethis will only piss them off and they will turn deeply invasive

Epithelioma cuniculatum (plantar surface of the foot)

on plantar foot

MALIGNANT LESIONS

Mycosis Fungoides (TCell) Pts are commonly misdiagnosed with eczema PRIMARY CUTANEOUS LYMPHOMAS

plaque stage: looks like eczema, but steroids don't work tumor stage: knee picture. Looks pretty bad

Sezary Syndrome

Leukemic form of Mycosis Fungoides high mortality rate

Paget's of Breast

Extension of underlying ductal carcinoma to the skin including the nipple, and areola Similar to Bowens dz MC labia majora F>M Associated with underlying malignancy 20% of the time -> most common is rectal, bladder, renal, uterine If you see a lesion and its associated with an internal cancer, 50% have already metastasized early onset of disease states: 1000x risk of skin cancers defect in nucleotide excision repair genes--> exaggerated response to UV

Surgery

PAGET DISEASE

Extramammary Paget's

XERODERMA PIGMENTOSUM

OCULOCUTANEOUS ALBINISM

pigment disorder of eyes, hair, skin

no melanin --> no UV protection --> inc SCC, melanomas > BCC

UV protection

EPIDERMODYSPLASIA VERRUCIFORMIS

flat wart-like lesions in early childhood --> acutally SCC

HPV 3, 5, 8

RECESSIVE DYSTROPHIC EB

basement membrane familial disease: skin sloughs off easily and SCC develops in scars or sites of chronic irritation

collagen type VII defect leading cause of death beyond childhood in pts w/ dystrophic EB --> septic SCC occurs on extremities and metastasizes early

BAZEX SYNDROME

ROMBO SYNDROME

early onset BCC follicular atrophoderma congenital hypotrichosis: loss of eyebrows and hair early onset BCC peripheral vasodilation w/ cyanosis hypotrichosis milium/cysts blepharitis