Abstract
Atrichia with papular lesions is a rare Autosomal recessive disorder characterized clinically by complete and irreversible hair loss shortly after birth, and is associated with the development of keratinfilled cysts all over the body. We present a 10 year old girl with extensive alopecia and papular lesions over scalp,upper limbs,lower limbs, abdomen and back . A clinical diagnosis of Atrichia with papular lesions was made after excluding Vit D dependent Rickets. An accurate diagnosis is important to prevent unnecessary treatment of APL with systemic steroids when misdiagnosed as alopecia universalis.
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