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

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

Synonyms: PLACK syndrome

A rare genetic skin disease characterized by generalized skin peeling or superficial blisters without scarring leukonychia acral punctate keratoses coalescing into focal keratoderma on the weight-bearing areas painful 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 is not specific and shows hyperkeratosis acanthosis and occasional intraepidermal clefting with irregular acantholysis.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.

Reference: Access aggregated data from Orphanet at Orphadata.

Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version April 2024

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