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.
Keyword :
Atrichia Congenita, Congenital Hairloss, Genodermatoses