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December 1989

Hidrotic Ectodermal Dysplasia With Diffuse Eccrine Syringofibroadenomatosis

Author Affiliations

Department of Dermatology

University of Turin Via Cherasco 23 10126 Turin, Italy

Arch Dermatol. 1989;125(12):1715. doi:10.1001/archderm.1989.01670240117032

To the Editor.—  Hidrotic ectodermal dysplasia (HED) is an uncommon autosomal-dominant disorder characterized by palmoplantar hyperkeratosis, dystrophy of the nails, and defects of the hair. It is sometimes associated with a large constellation of other abnormalities.1 In this report, we describe a case of HED in a patient with numerous papules on the palmoplantar surfaces, which revealed the histologic pattern of the eccrine syringofibroadenoma.

Report of a Case.—  A 53-year-old woman presented in our clinic with a thickening of palms and soles, hypotrichosis, and defects of the nails that had been present since infancy. For at least 2 years she had noticed papular lesions on the soles and palms. She was the fourth of five siblings of nonconsanguineous parents. Only her elder brother, 63 years of age, exhibited a similar condition, but he refused to undergo the skin biopsies. She told us that her teeth were conical, small, and