Mal de Meleda : a case successfully treated with acitretin

Abstract

Mal de Meleda (MdM) is an autosomal recessive form of palmoplantar keratoderma, that is characterized by transgradient keratoderma with associated scleroatrophy, nail changes, pseudoainhum around digits and perioral erythema, without a tendency for spontaneous resolution. Mal de Meleda can lead to severe flexion contractures in some patients as well as mycotic over-rinfections with significant impact on quality of life and daily activity. Mal de Meleda is a rare genetic disorder but no standardized treatment has been established. Treatment options for this disease include topical keratolytic agents, propylene glycol, topical 5-fluorouracil and surgical treatment. In addition, it has been reported that etretinate and acitretin, usually produce improvement. Herein, we present a case of Mal de Meleda with lips involvement which showed significant clinical improvement with acitritin.

Keywords

• mal de meleda
• acitretin
• palmoplantar
• keratoderma