Olmsted syndrome--congenital palmoplantar and periorificial keratoderma.

We are reporting the third case of the Olmsted syndrome, an entity that has received little attention in the medical literature. The syndrome consists of congenital diffuse, sharply marginated keratoderma of the palms and soles, onychodystrophy , constrictions of digits, and periorificial keratoses. Our patient, in addition, had congenital universal alopecia, absence of a premolar tooth, leukokeratosis of oral tissue, and hyperlaxity of the joints. The differential diagnosis includes hidrotic ectodermal dysplasia of the Clouston type, pachyonychia congenita, acrodermatitis enteropathica, keratoma hereditarium mutilans of Vohwinkel , mal de Meleda , and other forms of palmoplantar keratodermas . This patient had originally been reported as having acrodermatitis enteropathica, a condition with which the Olmsted syndrome can easily be confused.