A 24‐year‐old white man presented to our hospital with ulcerative skin lesions and scleritis. He had a history of relapsing polychondritis (RP) that was diagnosed 3 years back and was well controlled for last 1 year with azathioprine 175 mg daily and prednisone 2.5 mg daily. A month prior to his current admission he developed a flare of left eye scleritis and several mucocutaneous lesions. At that time he was admitted to outside hospital and was treated with i.v. steroids and methotrexate. During his hospital course his skin lesions worsened especially in the areas of his IV sites which was suggestive of pathergy reaction. Endoscopy revealed ulcers over left tonsil and tonsillar wall. As he failed to respond to treatment he was transferred to our hospital for further management. On arrival to our hospital his vital signs were: temp 36.3, BP 136/81, HR 68/minute RR 18/minute. Physical examination was significant for left eye conjuctival injection and multiple diffuse ulcerated lesions seen over both arms, forearms, right hand, chest, back, left buttock and thighs. WBCs were high at 26,000, CRP was elevated at 13.2. ESR was 52. An ophthalmologic evaluation revealed severe nodular scleritis and peripheral ulcerative keratitis of left eye. Extensive infectious workup including bacterial, viral, and fungal cultures were negative. Given the clinical picture compelling for new‐onset of Behcet's disease (BD), patient was treated with three days of methylprednisolone 1 g/day. His skin lesions improved subsequently. However on completing the pulse‐dose steroids, patient again had rapid deterioration of his keratitis with worsening epithelial defect with a concern of corneal melt. Given the extreme severity and the threat to his vision, patient was started on cyclophosphamide 150 mg daily. After the addition of cyclophosphamide, patient's eye condition improved. It was found that patient was sensitive not only to the dosage of steroids but also to the frequency of administration. Inflammation of the sclera and cornea finally improved with the use of prednisone 40 mg 3 times a day and cyclophosphamide 175 mg daily. His skin lesions healed well without development of any new lesions or pathergy to phlebotomy.
MAGIC (mouth and genital ulcers with inflamed cartilage) syndrome is a rare rheumatic disease that features the concurrence of RP/BD that was first described by Firestein et al. in 1985. Twenty‐two cases have been reported throughout the world since then. We described a patient with MAGIC syndrome and severe peripheral ulcerative keratitis threatening the vision that was refractory to methotrexate, azathioprine, and improved with cyclophosphamide and high‐dose steroids.
The presence of ulcerative keratitis and mucocutaneous lesions in cases of relapsing polychondritis should prompt considering a diagnosis of MAGIC syndrome. Institution of high‐dose steroids along with cyclophosphamide is an effective treatment approach.
To cite this abstract:Du L, Anumandla A, Kistangari G. Magic Syndrome with Vision‐Threatening Keratitis. Abstract published at Hospital Medicine 2013, May 16-19, National Harbor, Md. Abstract 418. Journal of Hospital Medicine. 2013; 8 (suppl 2). https://www.shmabstracts.com/abstract/magic-syndrome-with-visionthreatening-keratitis/. Accessed September 15, 2019.