Atrichia With Papular Lesions – A Case Report

Atrichia with papular lesions (APL) is a rare autosomal recessive disorder characterized by the loss of scalp and body hair shortly after birth, which is not replaced by terminal hair, and the development of follicular papules. This case report described a 4-year-old female with APL, presenting with hair loss and multiple keratotic papules. Despite normal hematological and biochemical parameters, histopathology showed remnants of hair follicles with perifollicular lymphocytic infiltrate and fibrosis. Treatment with topical tretinoin, tacrolimus, and biotin supplementation showed no improvement. The diagnosis was confirmed based on clinical and molecular criteria, including a family history of consanguinity and a mutation in the hairless gene. The report emphasized the importance of accurate diagnosis to avoid unnecessary treatments, as no effective treatment exists to stimulate hair growth in APL.