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MAGIC syndrome
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MAGIC syndrome

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Contributors: Paul Curtiss, Gabriela Cobos MD, Susan Burgin MD
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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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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Granulomatosis with polyangiitis (Wegener granulomatosis) – May cause pleuritis, nonerosive arthritis, ocular disease (uveitis, scleritis), saddle nose deformity, and cutaneous vasculitis but is commonly associated with glomerulonephritis. Patients are ANCA positive, primarily c-ANCA (PR3-ANCA).
  • Polyarteritis nodosa – A medium vessel vasculitis with similar cutaneous and histopathologic findings to BD and MAGIC syndrome, including leukoclastic vasculitis and mixed panniculitis. Polyarteritis nodosa may be associated with viral syndromes, including hepatitis B, and does not cause chondritis.
  • Systemic lupus erythematosus (SLE) – May cause oral ulcers and is often associated with polyarthritis. SLE is typically associated with increased levels of circulating auto-antibodies including ANA, which is often negative or equivocal in MAGIC syndrome.
  • Inflammatory bowel disease (Crohn disease / ulcerative colitis) – May cause ocular disease (uveitis, retinal vasculitis), oral apthae, erythema nodosum, diarrhea, and elevated inflammatory markers (ESR / CRP). Patients typically have characteristic findings on colonoscopy. Significant chondritis is rare.
  • Reactive (Reiter) arthritis – Associated with polyarthritis, ocular lesions (conjunctivitis, uveitis, episcleritis), and oral and genital ulcers. Often follows acute gastrointestinal infection and is not associated with chondritis.
  • Syphilis – Caused by the organism Treponema pallidum, it is a common sexually transmitted infection. The chancre seen in primary syphilis may manifest as a painless genital or oral ulceration. Saddle nose deformity is a feature of untreated congenital syphilis. Manifestations of tertiary syphilis include gummas and proximal syphilitic aortitis. Painful, recurrent oral and genital ulcerations associated with ocular abnormalities are not common in syphilis.
  • Tuberculosis – Caused by the organism Mycobacterium tuberculosis, tuberculosis is endemic in many regions of the world. Acute miliary tuberculosis, erythema induratum, and lupus vulgaris limited to the auricle may mimic acneiform papules, erythema nodosum, and auricular chondritis, respectively. Tuberculosis is associated with pulmonary symptoms and a positive interferon-gamma release assay (QuantiFERON) or purified protein derivative (PPD) test.

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Therapy

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References

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Last Reviewed: 08/30/2017
Last Updated: 10/04/2017
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MAGIC syndrome
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MAGIC syndrome : Oral ulcers, Edema, Erythema, Female genital, Male genital, Tender skin lesion
Copyright © 2019 VisualDx®. All rights reserved.