Diagnosis: Nekam's disease
Description: Keratotic papules in a linear and reticulate pattern
Morphology: Red,scaly
Site: Thigh
Sex: M
Age: 53
Type: Clinical
Submitted By: Ian McColl
Differential DiagnosisHistory:
Neckam's disease is a rare non itchy keratotic disorder with the papules mainly seen in acral areas, thighs and forearms with associated seborrhoeic derrmatitis and genital or oral lesions and sometimes with nail dystrophy and palmoplantar keratoderma. The condition is aquired rather than genetic and very chronic and resistant to treatment. Oral retinoids and some UV lighjt probably work best but there is a case report of success with Efalizumab given over 16 weeks.
The histology is a lichenoid infiltrate with marked basal vacuolar changes, colloid body formation and some epidermal atrophy alternating with hyperplasia. The infiltrate particularly involves the acrosyringium of the eccrine glands and there may be some epidermal plugging.
The condition is sometimes known as keratosis lichenoides chronicus