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Common adult causes
Clinical Clues
- Elderly men
- Chronic, relapsing course
- Pruritus +++
- Palmoplantar keratoderma
- Dermatopathic lymphadenopathy
Histologic clues
- Nonspecific
- Acute: parakeratosis, spongiosis, moderate lymphohistiocytic infiltrate
- Chronic: hyperkeratosis, psoriasiform acanthosis, expanded papillary dermis
Other clues
- Increased risk of recurrence
- Can develop into cutaneous T-cell lymphoma: re-evaluation with serial biopsies and blood flow cytometry indicated
Clinical Clues
- Pre-existing lesions
- Spares central face
- Nail changes: oil drop, pits, onycholysis
- Subcorneal pustules
- Inflammatory arthritis
Histologic clues
- Confluent/focal parakeratosis
- Epidermal hyperplasia with thin elongated rete ridges
- Epidermal neutrophils
- ↓ or absent granular layer
- Tortuous vessels in papillary dermis
Other clues
- Personal/family history of psoriasis
- Withdrawal of corticosteroids, methotrexate, cyclosporine, biologics
- Drug trigger, systemic infection or irritant contact dermatitis
- Allopurinol
- Β-lactam antibiotics
- Carbamazepine
- Gold
- Phenobarbital
- Phenytoins
- Sulfasalazine
- Sulfonamides
Clinical Clues
- Preceding morbilliform or scarlatiniform exanthem
- Facial edema
- Purpura on dependent areas
- Exfoliative (peeling) scale
- Drug rash with eosinophilia and systemic symptoms/Drug-induced hypersensitivity syndrome; fever, facial edema, peripheral eosinophilia, hepatitis, peripheral lymphadenopathy
Histologic clues
- Pan-epidermal apoptotic keratinocytes
- Perivascular infiltrate with eosinophils
- Many possible patterns: lichenoid, lymphomatoid, spongiotic, interface, drug rash with eosinophilia and systemic symptoms/drug-induced hypersensitivity syndrome
Other clues
- No history of skin disease
- Acute onset
- Resolves 2-6 weeks post cessation of drug trigger
- More frequent in human immunodeficiency virus
Clinical Clues
- Pre-existing lesions
- Pruritus +++
- Excoriations
- Lichenification
- Prurigo nodularis
- Fine scale
Histologic clues
- Parakeratosis
- Mild to moderate acanthosis
- Variable spongiosis and dermal edema
- Dermal eosinophils
- Variable lymphohistiocytic infiltrate with exocytosis of lymphocytes
Other clues
- Personal/family history of atopy
- High serum IgE, eosinophilia
- Cataracts/keratoconus
- Other stigmata of atopic dermatitis
Clinical Clues
- Pruritus +++
- Excoriations
- Lichenification
- Prurigo nodularis
- Melanoerythroderma
- Infiltration/edema
- Leonine facies
- Palmoplantar keratoderma with painful fissures
- Alopecia
- Lymphadenopathy
- Splenomegaly
Histologic clues
- Cerebriform pleomorphic lymphocytes
- Clustering of atypical cells in the epidermis
- Lichenoid infiltrate
- Histology is non-diagnostic in 30% of patients with Sézary syndrome
Other clues
- Sézary syndrome
European Organization of Research and Treatment of Cancer criteria:
-
- Erythroderma
- Clonal T-cell population in the blood and skinPlus 1 of the following
-
- ≥ 1000 Sézary cells/mm3
- CD4:CD8 ≥ 10:1
- Increased percentage of CD4+ cells with abnormal phenotype
- ≥ 40% CD4+/CD7–
- ≥ 30% CD4+/CD26