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When Tissue Is Not the Issue: A Case of Antisynthetase Syndrome Complicated by Disseminated Mycobacterium avium Complex.

A ntisynthetase syndrome is a rare autoimmune disease characterized by variable expression of fever, myositis, interstitial lung disease, arthritis, Raynaud phenomenon, and distinctive dermatologic features. Opportunistic infections are commonwith this condition and can present with nonspecific symptoms, which are difficult to differentiate from the underlying rheumatic disease. We present a patient with antisynthetase syndromewho developed constitutional symptoms, eyelid nodules with heliotrope rash, and tender soft tissue masses of the extremities. Initial biopsy of these masses was favored to be panniculitis secondary to dermatomyositis (DM), which will generally present in a lobular pattern with a lymphocytic predominant inflammatory infiltrate and mucin deposition in the deep dermis. The histology of these soft tissue lesions, coupled with persistently negative cultures, created considerable diagnostic uncertainty in a patient ultimately found to have a disseminatedMycobacterium avium complex (MAC) infection. A high level of clinical suspicion is required in cases such as this to avoid inappropriate escalation of immunosuppression. A 33-year-old woman with a 6-year history of anti-PL12– positive antisynthetase syndrome presented with tender palpable masses of her right posterior and lateral thigh. Her autoimmune disease, which had initially manifested as myositis, arthritis, fever, interstitial lung disease, Raynaud phenomenon, heliotrope rash, and Gottron's papules, had recently been well controlled on lowdose azathioprine and prednisone. She endorsed 2 months of subjective fevers, chills, night sweats, and a 20-pound weight loss. Physical examination was notable for 2 new erythematous eyelid nodules overlying her chronic heliotrope rash (Figs. 1A–D). A punch biopsy of the right posterior thigh revealed a lobular panniculitis with mixed inflammatory infiltrate and increased mucin in the deep dermis, favoring panniculitis related to DM (Fig. 2). Despite these histologic findings, a high level of concern for infection remained and further evaluation was pursued. Magnetic resonance imaging (MRI) and computed tomography