[Congenital atrichia with cysts]

A Delprat, J L Bonafé, Y Lugardon
Annales de Dermatologie et de Vénéréologie 1994, 121 (11): 802-4

INTRODUCTION: Congenital atrichia with papular lesions is a rare congenital ectodermic dysplasia.

CASE REPORT: A male child had atrichia of the scalp and eyebrows from birth. The atrichia was associated with epidermic cystic lesions of the scalp. Biopsy showed cystic piler follicles within the derma surrounded with an inflammatory infiltration of multinucleated giant cells.

DISCUSSION: In most reported cases, scalp hair is present at birth and then falls within the first three months of life. Follicular cysts appear later. This syndrome generally occurs sporadically and the mode of inheritance is unknown.

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