Calcinosis Universalis in Juvenile Dermatomyositis

A 12-year-old girl presented with a facial erythematous rash and limited range of motion of the elbow joint. She complained of swelling and limitation of the motion of both elbow joints. This patient presented with a history of juvenile dermatomyositis since the age of 5. She had maintained muscle strength, but had a waxing and waning skin rash, despite receiving treatment with multiple medications, including corticosteroids, intravenous immunoglobulin, methotrexate, and mycophenolate mofetil for over 6 years at our hospital.

On physical examination, she presented with diffuse palpable subcutaneous nodules in both the axilla and the upper extremities, erythematous changes, and shallow ulceration of the skin, overlying chin, and anterior neck area (Fig. 1A). There were multiple tender nodular lesions on the right axillar and a limited range of motion due to pain (Fig. 1B, C). Extrusion of white, crumbly matters in the vicinity of the posterior elbow were observed (Fig. 1D). A radiograph of the humerus revealed extensive calcification in both axillary areas, extending downwards (Fig. 2A). Magnetic resonance imaging revealed huge calcification and fluid collection in whole teres major, biceps brachii, brachialis and proximal pronator teres muscles (Fig. 2B, C).

FIG. 1. (A) Erythematous changes and shallow ulceration of the skin, overlying chin and anterior neck area. (B) Multiple nodular lesion on right axilla. (C) Limited range of motion on right elbow joint. (D) Extrusion of white, crumbly matter on right posterior elbow.

FIG. 2. (A) A radiograph of the humerus revealed clumped masses and sheet-like calcification in axilla and upper limbs. (B) Sagittal T2 MR image shows nodular calcifications (circle) as dark signal masses in right axilla, (C) sheet-like calcification (arrow) with hyper-intensity in muscle fibers suggest edema and inflammation in right upper limbs.

Given the patient's calcinosis universalis, she was treated with infliximab, intravenous bisphosphonate, and aluminum hydroxide.

Juvenile Dermatomyositis (JDM) is a rare autoimmune inflammatory disease of the muscles, affecting children and adolescents, presenting with soft tissue calcification and varying systemic involvement. Calcifications in JDM usually develop in the chronic phase at the sites of necrosis and involve the skin, subcutaneous tissue, fascial planes and muscles.1 Calcinosis in JDM patients can cause more incapacity and complications than the myopathy itself.2 Soft tissue calcification can be anywhere, including superficial skin (calcinosis cutis), around joints (calcinosis circumscripta) or deep fascial planes (calcinosis universalis).

There are four patterns of calcification described with dermatomyositis cases namely: superficial nodular-like calcified masses, deep calcified masses, deep sheet-like calcifications within the fascial planes, and a rare, diffuse, superficial lacy and reticular calcification which involves almost the entire body surface. The last pattern has been linked with a chronic, severe, non-remitting course, in patients with dermatomyositis, and needs to be differentiated from myositis ossificans progressive.3 The calcinosis in dermatomyositis is essentially a dystrophic calcification without formation of ossification, hence no evidence of zoning is seen in these calcified masses unlike in myositis ossificans progressive.4

To the best of our knowledge, this is the first report of Calcinosis Universalis complicating JDM in Korea.