[Congenital atrichia associated with keratin cysts - Variant of partial ectodermal dysplasia]

This is a report of three male siblings suffering from almost complete Atrichia, dental and osseous anomalies and also multiple disseminated horny papules. These latter lesions are caused by follicular plugging with a horny substance possibly due to a disorder of keratinisation as seen in patients with partial ectodermal dysplasia. Clinical manifestations and possible variations of this syndrome are described and the hereditary patterns are discussed.