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May 1979

Mal de Meleda Treated With 13-cis Retinoic Acid

Author Affiliations

From the Department of Dermatology, Division of Dermatopharmacology, New York University School of Medicine, New York (Drs Reed, Stanley, Stengel, and Shupack), and Department of Medical Research, Hoffmann-LaRoche, Inc, Nutley, NJ (Dr Benjamin).

Arch Dermatol. 1979;115(5):605-608. doi:10.1001/archderm.1979.04010050039014

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.

(Arch Dermatol 115:605-608, 1979)