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April 1994

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

Author Affiliations

Istituto di Clinica Dermatologica Università degli Studi di Bologna Via Massarenti, 1 40138 Bologna, Italy

Bologna, Italy

Milan, Italy

Bologna, Italy

Arch Dermatol. 1994;130(4):522-524. doi:10.1001/archderm.1994.01690040130027

Hereditary woolly hair has occasionally been associated with abnormalities of the skin,1 eyes, and ears. Barker et al2 have 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

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