Keratosis pilaris atrophicans faciei (KPAF), also known as ulerythema ophryogenes, is a disorder of follicular keratinization presenting in infancy. Inheritance is thought to be autosomal dominant with incomplete penetrance, although autosomal recessive and sporadic cases have been described. Genetic mutations in DSG-4, LRP-1, and MBTPS-2 have been implicated in some cases. KPAF is considered rare and has a male predilection. It is usually diagnosed in childhood or adolescence.

Affected individuals have keratotic papules along the eyebrows and cheeks beginning in the first few months of life, which can progress into scarring alopecia. Thinning or loss of the lateral eyebrows is a common finding. KPAF is often seen with keratosis pilaris on the arms and legs. Progression of the disease usually ceases at puberty, but sequelae are often permanent.

KPAF has been described in patients with woolly hair, Noonan syndrome, cardiofaciocutaneous syndrome, Rubinstein-Taybi syndrome, Cornelia de Lange syndrome, monosomy 18p, and Swyer syndrome. These patients will have additional features suggestive of these syndromes and may require workup and genetic evaluation.