Papular atrichia: report of 3 cases with uncommon presentation

Abstract

Atrichia with papular lesions (APL) is a rare, autosomal recessive syndrome with total alopecia in which mutations in the hairless gene have been shown to underlie the phenotype. We report here three cases, two 16 and 18 years old cousins and a 15 year-old-girl with a history of shedding of scalp, eyebrow and eye lashes in infancy. Later in life they developed numerous erythematous papules on their limbs predominantly on the knees and elbows. According to their history, clinical presentation and pathologic findings, diagnosis of atrichia with papular lesions (APL) with some different features was postulated. Comparing to the known cases of APL with scalp, facial and truncal lesions, the cases presented here do not show such lesions in these sites.