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June 2011

Congenital Hypertrichosis Lanuginosa in a Father and Son

Author Affiliations

Author Affiliations: Departments of Dermatology (Dr De Raeve) and Medical Genetics (Dr Keymolen); UZ Brussel, Vrije Universiteit Brussel, Brussels, Belgium.

Arch Dermatol. 2011;147(6):746-747. doi:10.1001/archdermatol.2011.137

Congenital hypertrichosis lanuginosa (CHL) is a rare disorder, with fewer than 50 cases reported in the literature. It is characterized by excessive lanugo hair present at birth covering the entire body surface except the mucosae, palms, and soles. It is considered to be an autosomal dominant disorder, but sporadic presentations are also reported. We report herein CHL in a father and son.

A 15-month-old boy presented with a history of excessive hairiness since birth that increased progressively during infancy. At presentation, he had excess long, dark blond, silky hair on the face, ears, trunk (Figure 1), and limbs (Figure 2). There was accentuation of the hairiness over the lumbosacral spine and sacrum (Figure 1). His eyebrows were very thick and coarse, his eyelashes surprisingly long. The face was coarse, and there was a small sacral dimple. There were no indications of skeletal abnormalities. Findings of ophthalmologic and stomatologic examinations were normal. Extensive laboratory analyses, including endocrinologic and metabolic evaluations, revealed no abnormalities. His karyotype was normal. Family history revealed that his father had also been unusually hairy during infancy. The father experienced severe dental caries and wore complete dental prostheses by age 27 years. The father was observed to have coarse eyebrows and long eyelashes at the time of his son's evaluation.