Mal de Meleda

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Synonym(s)

Acroerythrokeratoderma; keratoderma palmoplantaris transgradiens

Definition

Keratoderma of the palms and soles occuring as a genetic disease, mainly in residents of the island of Meleda

Pathogenesis

Recessive or variable dominant mode of transmission; exact genetic defect unknown

Clinical manifestation

Keratoderma of the palms and soles, with extension onto the dorsa of the affected limbs; extension to the elbows and knees; associated eczema in many patients; hyperhidrosis; nail thickening and koilonychia

Differential diagnosis

Olmsted syndrome; keratosis lichenoides chronica striata; mutilating keratoderma (Vohwinkel's syndrome); progressive palmoplantar keratoderma; tylosis (Unna-Thost syndrome)

Therapy

Keratolytic therapy, such as 6% salicylic acid in 70% propylene glycol; acetretin