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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.
