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Adult orbital xanthogranulomatous disease (AOXGD) is a rare granulomatous disorder. Adult-onset asthma with periocular xanthogranuloma (AAPOX) which is a subtype of AOXGD is very rare and a realtively unknown entity among dermatologists. Very few cases had been reported in literature. Clinically recurrent periorbital swelling and its location may mimic other dermatological conditions like sarcoidosis, necrobiotic xanthogranuloma, dermatomyositis, and rarely amyloidosis. Herein we report a female with recurrent periorbital swelling with brownish yellow papulonodular lesions on periorbital area with adult-onset asthma. Histopathology and immunohistochemistry proved the diagnosis. She was started with systemic methylprednisolone and methotrexate and improved significantly after 4 months. We report this case because of its rarity and to create awareness among dermatologists about this uncommon entity.

Adult-onset asthma with periocular xanthogranuloma (AAPOX), a variant of periorbital xanthogranulomatous disease: An uncommon entity