Alitretinoin: treatment for refractory palmoplantar keratoderma

DEAR EDITOR, Mal de Meleda (MDM) is an autosomal recessive form of palmoplantar keratoderma caused by mutations in the ARS gene, encoding SLURP-1. It has been reported that etretinate and acitretin, which are aromatic retinoids, are effective treatment modalities for MDM. However, early and long-term use of these retinoids are associated with several well-known adverse effects, such as dryness, teratogenicity and liver toxicity. A 20-year-old woman, diagnosed with MDM by identification of gene mutation in SLURP-1 in our previous report, presented with extensive palmoplantar hyperkeratosis which extended to the dorsal surfaces of her hands and feet from birth. She also complained of frequent recurrence of athlete’s foot with malodour. Previous treatments included keratolytic ointments, topical steroid, and topical and systemic antifungal agents. Over the past 30 months, oral acitretin, 10 or 20 mg daily, was administered additionally because of her increased cosmetic concerns. A modest improvement of her palmoplantar hyperkeratosis was observed, but most of the lesions did not show significant changes (Fig. 1a,b). Due to mucocutaneous discomfort and the need of a long period of contraception, acitretin was replaced by the new alitretinoin. After 3 months of treatment with alitretinoin 30 mg daily, her signs and symptoms improved significantly. Especially, the extent and thickness of hyperkeratosis were markedly reduced (Fig. 2a,b). In addition, she reported fewer mucocutaneous side-effects after drug replacement. Advanced lesions of MDM may show conical tapering of the fingertips, sometimes leading to spontaneous amputation