Introduction: Hereditary sclerosing poikiloderma is a genodermatosis with dominant autosomal transmission and variable penetration. The first case was described by Weary in 1969 in 7 members of two black families.
Case Report: A 10-year-old girl had localized regional poikiloderma of the fingers and club toes.
In 8 out of a series of 21 cases Dowling-Degos disease was associated with Verneuil's disease (chronic hidradenitis suppurativa). This association might be more common, since both diseases are characterized by a single defect (follicular occlusion) and occur in similar cutaneous areas.
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