J Rheum Dis.  2020 Oct;27(4):285-289. 10.4078/jrd.2020.27.4.285.

Dermatomyositis Presenting as Life-threatening Hypercalcemia

• Chiu HH
• Remalante PP
• Nacianceno R
• Velasco Jr R
• Larrazabal Jr R
• Zamora G

• KMID: 2506982
• DOI: http://doi.org/10.4078/jrd.2020.27.4.285

Abstract

Dermatomyositis is a rare disease characterized by classic skin lesions and muscle weakness. In rare cases, life-threatening hypercalcemia may develop caused by regression of dystrophic calcifications. Here we report a 36-year-old man who presented with progressive proximal weakness, difficulty in ambulation, and weight loss. He had the V-sign, Gottron’s papules, and hard, chalky nodules on both antecubital, thigh, and hip areas. Laboratory examinations revealed hypercalcemia (3.47 mmol/L) and shortened QT interval. Workup for malignancy and tuberculosis yielded negative results. Biopsy of the antecubital areas revealed calcinosis cutis. Serum calcium levels gradually normalized with hydration and steroids. Our case illustrated that a high index of suspicion for dermatomyositis is warranted for early diagnosis and ascertaining the etiology of hypercalcemia is vital in the management of this life-threatening complication. While hypercalcemia from dermatomyositis may respond to steroids, to date, individualization of treatment remains the standard of care.

Keyword

Dermatomyositis; Hypercalcemia; Calcinosis

Figure

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  Figure 1 (A) Multiple erythematous patches on the anterior neck and upper chest (V-sign). (B) Multiple hyperpigmented papules with areas of hyperkeratosis over the metacarpop-halangeal joints, with periungual discolor-ation of both hands. (C, D) Multiple hard nodules on the bilateral antecubital areas (calcinosis cutis). Informed consent for publication of clinical data and images was obtained from the patient prior to the beginning of the study.

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  Figure 2 Plain radiographs of the left elbow (A) and upper arm (B) showing multiple radioopaque areas of calcifications (ovals and rectangle).

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  Figure 3 Low-power field (×10 magnification) using H&E stain: A cylindrical skin segment biopsy of the right elbow: basophilic deposits (oval) in the dermis with surrounding cell infiltrates of lymphocytes, histiocytes and multinucleated giant cells (arrows).

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