Peeling skin-leukonychia-acral punctate keratoses-cheilitis-knuckle pads syndrome

Orpha code: 444138OMIM code: 616295

Definition

A rare genetic skin disease characterized by generalized skin peeling, leukonychia, acral punctate keratoses coalescing into focal keratoderma on the weight-bearing areas, angular cheilitis, and knuckle pads with multiple hyperkeratotic micropapules. The skin appears dry and scaly with superficial exfoliation and underlying erythema. Histopathologic examination of affected skin areas shows hyperkeratosis, acanthosis, and intraepidermal clefting with irregular acantholysis. Additional systemic abnormalities are absent.

Disease data
Classification

Disease

Synonyms
PLACK syndrome
Zespół plACK
ORPHA code
444138
OMIM code
616295
ICD10 code
Q82.8
ICD11 code
-

No additional description.

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