Abstract
Seven members of 1 family had a rare, inherited syndrome of follicular atrophoderma, basal cell carcinomas and localized anhidrosis. Associated findings in some subjects were generalized hypohidrosis and eczema involving the face shortly after birth. This syndrome shows dominant inheritance, possibly X-linked. Follicular atrophoderma is an important skin manifestation, as it was associated in all reported cases with other systemic or cutaneous abnormalities.

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