Diffuse nonepidermolytic palmoplantar keratoderma
Diffuse nonepidermolytic palmoplantar keratoderma
Definition
A rare, genetic, isolated diffuse palmoplantar keratoderma characterized by diffuse, mild to thick, finely demarcated hyperkeratosis of palms and soles. Additional clinical findings include knuckle pad-like keratoses on fingers, hyperkeratosis of umbilicus and areolae, diffuse dry skin, hyperhidrosis, hangnails and frequent fungal infections. Histological examination of lesions reveals orthokeratotic hyperkeratosis, acanthosis, hypergranulosis, and mild lymphocyte infiltrations in the upper dermis with no evidence of epidermolysis.
Also known as diffuse nonepidermolytic palmoplantar keratoderma, diffuse palmoplantar keratoderma, Bothnian type, KRT1-related diffuse nonepidermolytic keratoderma, NEPPK, non-epidermolytic palmoplantar keratoderma, nonepidermolytic palmoplantar keratoderma, palmoplantar keratoderma, nonepidermolytic — per MONDO