Mal de Meleda treated with 13-cis retinoic acid

Identical twins with mal de Meleda, a rare genodermatosis, displayed the characteristic "glove and sock" hyperkeratosis, hyperhidrosis, and malodor. Their parents, who are first cousins, are unaffected and originated from Calabria, Italy, which is not far from Meleda (Mljet). The disorder is probably transmitted as an autosomal recessive trait. Previous therapy with grenz rays, topical preparations of adrenocorticosteroids, lactic acid, retinoic acid, and bland emollients had been unsuccessful. One of the twins was treated with 13-cis retinoic acid taken by mouth for 16 weeks with dramatic improvement. The major adverse effect was cheilitis, which did not force discontinuation of the treatment.