This young man has all the signs of an extremely rare variant of keratosis pilaris (KP) called ulerythema ophryogenes (UO). In the United States, about 40% of adults have ordinary KP, which usually manifests in childhood (with about 80% of cases occurring in adolescent girls). KP is inherited through autosomal dominance, with highly variable penetrance, though no specific gene has been identified. In this form, KP is considered by most experts to be a minor diagnostic criterion for atopic dermatitis.
However, UO is not merely a variant of KP. Over the decades, it has been connected with more serious conditions, such as cardiofaciocutaneous syndrome, Rubinstein-Taybi syndrome, and Cornelia de Lange disease. Although these conditions are not common, they should be considered when UO is seen.
For this patient, the main concern was his appearance, especially the pronounced erythema around the periphery of his face. This aspect of the problem was addressed with a referral to a provider who can, using an assortment of lasers, try to even out his skin color and hopefully erase the sharp border at the periphery of the affected area.
For the physical discomfort caused by UO, the patient was instructed either to use general moisturizers to reduce dryness or to consider using moisturizers containing salicylic acid, which should help to reduce the prominence of the papules. For the erythema in his brows, he is using 2.5% hydrocortisone ointment two to three times a week.
TAKE-HOME LEARNING POINTS
- Ulerythema ophryogenes is a rarely encountered variant of keratosis pilaris—a condition inherited by autosomal dominance with highly variable penetrance.
- Its main significance, beyond cosmetic concerns, is the possible connection with syndromes that involve heart and structural defects (eg, cardiofaciocutaneous syndrome).
- Treatment options include heavy emollients to soften the scaly papules and laser therapy to reduce the extreme redness seen on the periphery of the face.