Cpc Kurban III
Transcript of Cpc Kurban III
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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
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Case 1
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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.
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Ofuji's Papuloerythroderma
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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.
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Case 2
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Case #1: Axilla
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Case #1: Axilla
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Case #1: Post. Cervical
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Case #1 Inguinal Folds
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distinct retention of basophilic keratohyaline granules within areas of parakeratosis in the stratum corneum
Axillary Granular Parakeratosis
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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
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Pathology
• Psoriasiform hyperplasia.
• Thickened stratum corneum with parakeratosis and retention of keratohyaline granules.
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(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.
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Case 3
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Mastocytosis
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• 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).
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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).
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Cuteanous Mastocytosis:• Solitary mastocytoma• Urticaria pigmentosa• Diffuse cutaneous mastoctosis• Telengectasia Macularis eruptiva Perstans
(TMEP).
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• Telangiectases and a very subtle increase in mast cell number around superficial vessels may be the only findings
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• Mast cells have round, dark nuclei and moderate surrounding grayish granular cytoplasm resembling "fried eggs
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• Toluidine blue stain confirms the diagnosis
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• Another helpful stain to confirm mast cells is chloroacetate esterase
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• Giemsa or toluidine blue stain shows metachromasia and amphophilic mast cell granules, confirming the diagnosis of mast cell disease
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• Electron microscopy shows characteristic electron-dense granules
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Differential Diagnosis
• Mastocytoma : Juvenile xanthogrnuloma, Spitz nevus
• UP, DCM, TMEP: histiocytosis X, secondary syphilis, papular sarcoid, generalized eruptive histiocytosis.
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Management
• Avoidence of drugs that cause mast cell degranulation
• Antihistamine• PUVA
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Case 4
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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
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PIEZOGENIC PEDAL PAPULES
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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
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• 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.
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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
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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.
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Differential Diagnosis
• Nevus lipomatosus superficialis• EDS
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Case 5
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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.
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Scleromyxedema
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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
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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
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Differential Diagnosis
• Folliculotropic mycosis fungoides• Scleroderma• Amyloidosis• Nephrogenic fibrosing dermopathy
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Treatment and Prognosis
• Physiotherapy• Systemic steroids• Retinoids, plasmapheresis, photopheresis• IVIG, EBT, PUVA, IFN, CyA, IL kenalog• Melphalan, cyclophosphamide• Autologous stem cell transplant • Prognosis poor
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Case 6
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Pigmented Purpuric DermatosisSchamberg's Disease
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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
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Case 7
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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)
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Case 8
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Mycosis fungoides/ CTCL
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Mycosis fungoides/ CTCL
Minimal to moderate spongiosis
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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
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Case 9
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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
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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
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Papillary dermal edema
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Neutrophils in their natural habitatNeutrophils in their natural habitat
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Dermatopathology Interactive Atlas, Bhawan et al.
NeutrophilsNeutrophils
PerivascularPerivascular NodularNodular DiffuseDiffuse
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Sweet’sSweet’s VasculitisVasculitis
Fibrinoid Necrosis
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Case 10
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Granuloma Faciale: Clinical
• Red/brown infiltrated plaques• Face/nose• Extrafacial sites: trunk,
extremities, scalp• White males, middle age
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Granuloma Faciale: Histology
• Normal epidermis• Grenz zone• nodular and/or diffuse
infiltrate in upper dermis• Eosinophils,
neutrophils; also lymphs and plasma cells
• Leukocytoclastic vasculitis
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eosinophil
neutrophil
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Granuloma Faciale
• LCV• Neutrophils
predominate
Dermatopathology Interactive Atlas, Bhawan et al.
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Case 11
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Secondary Syphilis: Clinical
• Papulosquamous eruption, “copper-colored”• Annular on face• non-pruritic• Acral: symmetric papules with colarrette of scale• Condyloma lata• Moth-eaten alopecia
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Secondary Syphilis: Histology• Psoriasiform hyperplasia• Hyperkeratosis and Parakeratosis• Spongiosis/Vaculolization at DE junction
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Secondary Syphilis: Infiltrate
PerivascularPerivascular NodularNodular LichenoidLichenoid
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Plasma Cells
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• Endothelial swelling with pseudo-granulomatous aggregates
• Silver stain (Warthin-Starry) shows spirochetes in epidermis
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Case 12
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Hidrocystoma
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Hidrocystomas
• 1 to 3mm translucent papules, occasionally with bluish tint
• Solitary-face, scalp
• Tx-excision, laser, atropine, scopolamine.
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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
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Case 13
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Chromomycosis
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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.
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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
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Case 14
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Syringocystadenoma Papilliferum
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Syringocystadenoma Papilliferum-clinical
• Commonly develop within nevus sebaceus of Jadassohn
• Rose-red papules of firm consistency.
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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
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Case 15
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Porphyria Cutanea Tarda
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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.
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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.
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