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MAGIC syndrome
Other Resources UpToDate PubMed

MAGIC syndrome

Contributors: Paul Curtiss MD, Gabriela Cobos MD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Mouth and genital ulcers with inflamed cartilage (MAGIC) syndrome is a rare autoimmune disease with fewer than 25 cases reported. It shares features of both Behçet disease (BD) and relapsing polychondritis (RP). It is characterized by autoimmune vasculitis with the potential to affect small, medium, and large vessels and erosive inflammatory disease of cartilaginous tissues. Patients commonly present with classic features of BD (oral / genital ulcers, ocular disease, cutaneous vasculitis) and, years later, develop early symptoms of RP (auricular chondritis and polyarthritis).

MAGIC syndrome typically occurs in the third decade of life but is described in patients ranging from ages 10-59. The majority of cases have been reported in the United States, Western Europe, and Japan. This differs from BD, which more commonly occurs in patients from the "Silk Road" involving parts of the Middle East, Central Asia, Eastern Europe, and Japan.

Given the protean manifestations of both BD and RP, patients with MAGIC syndrome show a varied disease course ranging from limited auricular chondritis with mucocutaneous ulcerations to significant end organ dysfunction. Described complications include hearing loss, pleuritis, gastrointestinal ulcerations, central nervous system (CNS) vasculitis, and deep vein thrombosis. Inflammatory aortitis is the most serious complication, which can be life threatening and occurs in approximately 25% of reported cases.

Codes

ICD10CM:
K13.70 – Unspecified lesions of oral mucosa

SNOMEDCT:
238850005 – Mouth and genital ulcers with inflamed cartilage syndrome

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Last Reviewed:08/29/2017
Last Updated:10/03/2017
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MAGIC syndrome
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A medical illustration showing key findings of MAGIC syndrome : Oral ulcers, Edema, Erythema, Female genital, Male genital, Tender skin lesion
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