Congenital atrichia with papular lesions

Abstract

Congenital alopecia has broad differential diagnosis and poses diagnostic and therapeutic challenges. It is a rare form of irreversible alopecia inherited autosomal recessively. Atrichia congenita with papular lesions represents a complex and heterogeneous group of genodermatoses characterized by irreversible complete hair loss soon after birth, and associated with the development of keratin-filled cysts over the body. We report a case of 4-year-old boy presenting with complete loss of hair over scalp, eye brows, eye lashes, and body since birth. Patient also had papular lesions over body.