Woolly Hair, Palmoplantar Keratoderma, and Cardiac Abnormalities: Report of a Family

Hereditary woolly hair has occasionally been associated with abnormalities of the skin,1eyes, and ears. Barker et al2have recently reported seven families in Greece affected by a new autosomal recessive syndrome characterized by the asso- ciation of woolly hair, palmoplantar keratoderma, and cardiac abnormalities.2 We describe two members of a family who presented a similar, but not identical, condition. Report of Cases. Case 1.A 20-year-old electrician was admitted to our clinic in June 1991 for a palmoplantar keratoderma. His history revealed that the plantar keratoderma had been present since the age of 1 year and had progressively worsened during the years. Palmar hyperkeratosis first appeared at the age of 17, when the patient started his job as an electrician. The clinical examination revealed a very thick, yellow hyperkeratosis over the pressure areas of the patient's palms and soles (Figure 1). Painful hyperkeratotic lesions of the