Sai Sreenivasulu Nookala

Abstract

Atrichia congenita with papular lesions is rare, autosomal recessive condition that leads to complete hairloss which is irreversible and with keratin filled papular lesions. An insertion mutation in the exon 2 of hairless gene is responsible for the hairloss. Middle and lower portions of hair follicle are replaced by keratinizing cysts with absence of hair shafts. We here by reporting a case of 8-year-old girl with complete hairloss over scalp, eyebrows, eyelashes, and body which started at birth and complete hairloss was gradual by 9 months age on view of its rarity and misdiagnosis.   Keywords: Atrichia Congenita, Congenital Hairloss, Genodermatoses

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