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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.
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:
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
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.