Papular umbilicated granuloma annulare in association with Alagille syndrome.

A 10-year-old white boy with Alagille syndrome was referred to the dermatology clinic for evaluation of an eruption on the lower extremities. The lesions were mildly pruritic, gradual in onset, and increased in number over several months. The patient reported no insect bites, no unusual contacts, no history of chronic dermatologic disorders, and no recent febrile illness or systemic complaints. He had Alagille syndrome with cholestatic liver disease, hyperlipidemia, pulmonary artery stenosis, retinal pigment abnormalities, and myopia. His medications included rifampin and phenobarbital. There was no family history of Alagille syndrome; however, the patient's father had retinal cone abnormalities and a half-sister had died of Aicardi syndrome. Physical examination revealed typical facies with hypertelorism, deep set eyes, and a sharply pointed chin. Dermatologic examination showed firm umbilicated papules symmetrically distributed on the dorsa of both feet and ankles (Fig. 1). The differential diagnosis included xanthomas, prurigo papules, and molluscum contagiosum. A skin biopsy revealed palisading necrobiotic granulomas consistent with granuloma annulare (Fig. 2).