Cpc Kurban III

CPC III• 1) Deckchair sign ( Ofuji's Papuloerythroderma)

• 2) Axillary Granular Parakeratosis

• 3) Mastocytosis• 4) PIEZOGENIC PEDAL

PAPULES

• 5) Scleromyxedema• 6) PPD• 7) PMLE• 8) CTCL

• 9) Sweet Syndrome• 10) Granuloma Fasciale• 11) Secondary Syphllis• 12) Hidrocystoma• 13) Chromomycosis• 14) Syringocystadenoma

Papilliferum• 15) PCT

Case 1

JAAD 2001 Iotaderma (#84)The “deck chair sign” refers to sparing of the creases in skin folds in an erythroderma consisting of confluent flat-topped pink papules and associated with a peripheral eosinophilia. What is the eponymic name for the disease in which the “deck chair sign” occurs?Answer: Papuloerythroderma of Ofuji

Ofuji S, Furukawa F, Miyachi Y, Ohno S. Papuloerythroderma. Dermatologica 1984;169:125-30.

Ofuji's Papuloerythroderma

Ofuji's Papuloerythroderma• Diffuse, papular erythroderma which spares the skin folds, creating the is characteristic ‘deckchair sign’ • Many of these patients have a peripheral eosinophilia, and some lymphadenopathy• Recently thought to be not a single entity but instead a pattern of expression of various inflammatory dermatoses, including lymphoma, hypereosinophilic syndrome, cancers, atopic dermatitis, tinea versicolor, and drug reactions• The work-up should include the exclusion of the above-mentioned entities, especially lymphoma.

Case 2

Case #1: Axilla

Case #1: Axilla

Case #1: Post. Cervical

Case #1 Inguinal Folds

distinct retention of basophilic keratohyaline granules within areas of parakeratosis in the stratum corneum

Axillary Granular Parakeratosis

Axillary Granular Parakeratosis• The primary lesions are brownish-red keratotic papules that can coalesce into plaques

• It occurs almost exclusively in women

• In most cases, lesions are localized to the axilla, but other intertriginous sites can be affected

• A defect in processing profilaggrin to filaggrin is a proposed mechanism

Pathology

• Psoriasiform hyperplasia.

• Thickened stratum corneum with parakeratosis and retention of keratohyaline granules.

(Axillary) Granular Parakeratosis

• Benign condition, with hyperpigmented and hyperkeratotic papules and plaques in the flexural folds

• Uncertain etiology: may be associated with use of topical agents, e.g. antiperspirants and occlusion

• Mostly affect women from 40-50 years of age, but may affected children as well.

• May associate with pruritus.• Effective treatment include topical and oral retinoids,topical

calcipotriene, and topical ammonium lactate.

Case 3

Mastocytosis

• It is a disease in which there is an increased number of mast cells in various organs of the body, the most frequent site of organ involvement is the skin.

• Mast cells contain : histamine (urticaria, Gi symptoms), prostaglandinD2 (flush, CVS, GI symptoms), heparin (bleeding into lesion at biopsy site), proteases, acid hydrolases (patch hepatic fibrosis and bone lesions).

• Stroking lesion causes it to itch and to wheal (Darier’s sign).

Triggers that induce systemic mast cell degranulation:

• Drugs (opiate analgesics, Vancomycin, Aspirin, NSAIDs, Muscle relaxants)

• Temperature changes : heat, cold.• Ingestion of alcohol.• Mechanical irritation : massage, Friction.• Infections (bacterial, Viral, Ascaris)• Insect stings, posion (snakes, Jellyfish).

Cuteanous Mastocytosis:• Solitary mastocytoma• Urticaria pigmentosa• Diffuse cutaneous mastoctosis• Telengectasia Macularis eruptiva Perstans

(TMEP).

• Telangiectases and a very subtle increase in mast cell number around superficial vessels may be the only findings

• Mast cells have round, dark nuclei and moderate surrounding grayish granular cytoplasm resembling "fried eggs

• Toluidine blue stain confirms the diagnosis

• Another helpful stain to confirm mast cells is chloroacetate esterase

• Giemsa or toluidine blue stain shows metachromasia and amphophilic mast cell granules, confirming the diagnosis of mast cell disease

• Electron microscopy shows characteristic electron-dense granules

Differential Diagnosis

• Mastocytoma : Juvenile xanthogrnuloma, Spitz nevus

• UP, DCM, TMEP: histiocytosis X, secondary syphilis, papular sarcoid, generalized eruptive histiocytosis.

Management

• Avoidence of drugs that cause mast cell degranulation

• Antihistamine• PUVA

Case 4

Histology• Marked decrease in the thickness of the dermis • Normal epidermis • Abnormal collagen fibers arranged in thin fibers

rather than in bundles • Subcutaneous fat extends through the dermis

and encroaches on the epidermis in some areas• Thin collagen fibers : seen bt. the lobules of

subepidermal adipose tissue. • EM: fine filamentous structures , normal-

appearing collagen fibers

PIEZOGENIC PEDAL PAPULES

PIEZOGENIC PEDAL PAPULES

• herniation of fat through the dermis. • Common• Non-hereditary• not the result of an inherent connective

tissue defect• Rarely found asso. with EDS

• No racial predisposition• Sex: women ( obesity) > men• Age: any age• asymptomatic • No treatment required• If the condition is painful, patients may

report limitation of occupational or sporting activities.

Features

• Skin color, compressible papule• Common : lateral heels,bilaterally; volar

wrists • Examine patients standing with their full

weight on the heels.• Papules resolve when the weight is

removed

Causes

• No specific • believed to be sporadic. • more common overweight, prople with

orthopedic problems ( flat feet), & may occur more commonly in persons with collagen disorders such as EDS.

Differential Diagnosis

• Nevus lipomatosus superficialis• EDS

Case 5

Histopathology of Scleromyxedema

Typical triad of - fibrosis - proliferation of irregularly arranged

fibroblasts - interstitial deposits of mucin in the upper

and mid-reticular dermis. : Mucin deposits splay collagen bundles in

the dermis, but there is only slight fibroblast proliferation and no sclerosis.

Scleromyxedema

Scleromyxedema

• = Generalized papular mucinosis• Adults, M=F• Chronic, progressive, pruritic• Multiple waxy/shiny papules, coalesce into

plaques• Dorsal hands, face, elbows, ears, extensor

extremities, leonine facies• Doughnut sign• Visceral: GI, pulm., musculoskeletal, CNS

Differential Diagnosis

• Mucin deposition• Fibroblast proliferation• Fibrosis• Normal thyroid function tests• Monoclonal gammopathy, usually IgGλ

type• Bone marrow: N or incr. plasma cells, or

myeloma

Differential Diagnosis

• Folliculotropic mycosis fungoides• Scleroderma• Amyloidosis• Nephrogenic fibrosing dermopathy

Treatment and Prognosis

• Physiotherapy• Systemic steroids• Retinoids, plasmapheresis, photopheresis• IVIG, EBT, PUVA, IFN, CyA, IL kenalog• Melphalan, cyclophosphamide• Autologous stem cell transplant • Prognosis poor

Case 6

Pigmented Purpuric DermatosisSchamberg's Disease

Pigmented Purpuric DermatosesSynonyms:

Capillaritis; Purpura pigmentosa chronica

Variants:• Progressive pigmentary dermatosis of Schamberg; Schamberg's disease

• Purpura annularis telangiectodes of Majocchi; Majocchi's disease

• Pigmented purpuric lichenoid dermatitis of Gougerot and Blum

• Eczematid-like purpura of Doucas and Kapetanakis

• Lichen aureus UNIFYING KEY FEATURES: • Clustered petechial hemorrhage• Often a background of yellow-brown discoloration due to hemosiderin deposition

Case 7

Polymorphous light eruption• Pseudobulla:

Marked papillary edema

• Tight superficial and deep lymph. infiltrate

• Occasional dyskeratosis and exocytosis • DDx: Pernio (acral skin with milder edema, more diffuse infiltrate)

Case 8

Mycosis fungoides/ CTCL

Mycosis fungoides/ CTCL

Minimal to moderate spongiosis

Mycosis fungoides/ CTCL

• Lymphocytes adhere to basal layer but do not obscure it

• Lymphocytes may have halos, are not perfectly round and may be slightly enlarged

• microabscesses may not be present

• degree of atypia is variable, may be low

Case 9

Sweets Syndrome: ClinicalAcute Febrile Neutrophilic Dermatosis

• Erythematous plaques; can be bullous

• Face, neck, upper extremities• Uncommon; Female > Male 4:1• Age 30-60 yrs• Possibly hypersensitivity reaction or

cytokine dysregulation• Idiopathic, paraneoplastic (20%), IBD/

autoimmune, drugs, and pregnancy• Associated fever, leukocytosis,

arthritis/arthralgias, ocular, pulmonary, renal, hepatitis

Sweet’s Syndrome: Histology• Edema of the papillary dermis,

severe (can form blister)• Dense neutrophilic infiltrate• Leukocytoclasia (but no

vasculitis)• Epidermal hyperplasia• Epidermal neutrophilic

microabcesses• Vascular ectasia

Papillary dermal edema

Neutrophils in their natural habitatNeutrophils in their natural habitat

Dermatopathology Interactive Atlas, Bhawan et al.

NeutrophilsNeutrophils

PerivascularPerivascular NodularNodular DiffuseDiffuse

Sweet’sSweet’s VasculitisVasculitis

Fibrinoid Necrosis

Case 10

Granuloma Faciale: Clinical

• Red/brown infiltrated plaques• Face/nose• Extrafacial sites: trunk,

extremities, scalp• White males, middle age

Granuloma Faciale: Histology

• Normal epidermis• Grenz zone• nodular and/or diffuse

infiltrate in upper dermis• Eosinophils,

neutrophils; also lymphs and plasma cells

• Leukocytoclastic vasculitis

eosinophil

neutrophil

Granuloma Faciale

• LCV• Neutrophils

predominate

Dermatopathology Interactive Atlas, Bhawan et al.

Case 11

Secondary Syphilis: Clinical

• Papulosquamous eruption, “copper-colored”• Annular on face• non-pruritic• Acral: symmetric papules with colarrette of scale• Condyloma lata• Moth-eaten alopecia

Secondary Syphilis: Histology• Psoriasiform hyperplasia• Hyperkeratosis and Parakeratosis• Spongiosis/Vaculolization at DE junction

Secondary Syphilis: Infiltrate

PerivascularPerivascular NodularNodular LichenoidLichenoid

Plasma Cells

• Endothelial swelling with pseudo-granulomatous aggregates

• Silver stain (Warthin-Starry) shows spirochetes in epidermis

Case 12

Hidrocystoma

Hidrocystomas

• 1 to 3mm translucent papules, occasionally with bluish tint

• Solitary-face, scalp

• Tx-excision, laser, atropine, scopolamine.

Path: Hidrocystoma

• Cyst-may appear empty from fluid running out in processing.

• Cyst lining gives diagnosis-– Cuboidal possibly bilayered-eccrine– Columnar possibly bilayered with decapitated

secretion-apocrine

Case 13

Chromomycosis

Clinical-chromomycosis

• Usu lower extremities, 4:1 men (farmers)• Verrucous, slow growing, 15 yrs to

diagnosis• Dematiacoius fungi-Fonsecaea pedrosoi

most common.• Tx: excision, cryo, itraconazole,

terbinafine.

Path: Chromomycosis

• Pseudoepitheliomatous hyperplasia, occ intraepidermal neutrophilic abscess

• Mixed dermal infiltrate-neuts, lymphs, histiocytes, plasma, and giant cells.

• Cluster or chains of brown spores-copper pennies, medlar bodies

Case 14

Syringocystadenoma Papilliferum

Syringocystadenoma Papilliferum-clinical

• Commonly develop within nevus sebaceus of Jadassohn

• Rose-red papules of firm consistency.

Path: Syringocystadenoma Papilliferum

• Papillomatous epidermis connecting to underlying tumor

• Cystic space in tumor opens to skin.• Superficial tumor lined by squamous epithelium,

deeper-sweat gland epithelium.• Apocrine decapitation usually present• Plasma cell infiltrate• Nevus sebaceus usually present

Case 15

Porphyria Cutanea Tarda

Clinical PCT• Photosensitivity resulting in bullae-sun exposed.• No erythema surrounding-rupture to ulcers.• Hyperpigmentation and hypertrichosis is often

seen.• Associated with liver disease or estrogen

therapy.• Deficiency in uroporphyrinogen decarboxylase.• Tx: antimalarials and phlebotomy.

Path-PCT

• Subepidermal blister with festooning of dermal papillae.

• Caterpillar bodies-eosinophilic, linear, segmented, basement membrane material resembling dyskeratotic cells.

• Sparse hyalizined material around vessels.• DFI-IgG and C3 around papillary dermal

vessels.