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Febrile Calcinosis in Scleroderma

Right arm extensor surface (A) and left arm volar surface (C) prior to steroid therapy, depicting mottled erythema together with subcutaneous calcific deposits at the volar aspect of the upper arm, forearm, and antecubital fossa. Right arm (B) and left arm (D) after initiation of high-dose steroid therapy, showing marked improvement and resolution in erythema.

The collagen vascular diseases can be challenging for the internist and are associated with higher utilization of resources and less favorable outcomes among patients admitted to the inpatient medicine services.1 In this context, the acquisition of proficiency in recognizing a range of disease manifestations, common and uncommon, and familiarity with the appropriate therapeutic interventions are critical skills to be enhanced.2 In this report, we highlight the challenging diagnostic and therapeutic predicament, centered around daily high spiking fevers, of an enigmatic patient whose hospital course achieved a favorable outcome when the etiology of the fever was reformulated and managed in a novel manner.

A 67-year-old woman was hospitalized with fever of 39°C (102.4°F), chills, and sweats, together with bilateral arm pain of 72 hours’ duration. Her decade-long history of limited scleroderma included sclerodactyly, telangiectasia, Raynaud’s phenomenon, and calcinosis. At disease onset, a heliotrope rash and skin biopsy demonstrated interface dermatitis, suggesting overlapping features with dermatomyositis.

Examination revealed an alert, interactive yet diaphoretic woman. There was mottled erythema of both arms, with substantial pain limiting shoulder and elbow motion (Figure 1). Calcinosis deposits at the volar aspect of the upper arms and forearms were exquisitely tender. A leukocytosis of 18.57 k cells/mm3, anemia with hemoglobin of 8.4 g/dL, and mild thrombocytosis at 435 k platelets/mm3 were noted. Erythrocyte sedimentation rate was 128 mm/hour, and C-reactive protein was 31 mg/L. Renal function was normal.

Broad-spectrum antimicrobial therapy to treat presumptive cellulitis was commenced; however, after 5 days of empiric treatment, there was no resolution in symptoms or signs, with ongoing daily fever spikes to 38.2°C (100.8°F) or greater. Moreover, x-ray and computerized tomography imaging of the arm (Figure 2) demonstrated dermal thickening and soft-tissue calcinosis without abscess. Although it is uncharacteristic for scleroderma to induce fever, we speculated that the calcinosis might be inciting a febrile inflammatory response via crystal-mediated cytokine release. Methylprednisolone dosed at 1 mg/kg/day intravenously for 3 consecutive days was begun. After the first dose of steroid medication, she promptly defervesced, and there was noticeable improvement in her arm pain and swelling (Figure 1).

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-Allan C. Gelber, MD, Michael I. Brener, MD, Kaelin O’Connell, MD, Michael Wu, MD, Julie J. Paik, MD, Jemima F. Albayda, MD

This article originally appeared in the December issue of The American Journal of Medicine.

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