Calcinosis in juvenile dermatomyositis

Abstract

Abstract.A retrospective study of the radiographs and clinical records of 39 children with dermatomyositis, followed up at a referral centre for a mean period of eight years, revealed calcinosis in 29 children (18 girls, 11 boys). Clinical presentation of calcinosis was unpredictable and variable, ranging from four months to 12 years after onset of disease. Subcutaneous calcification, often associated with subsequent ulceration of overlying skin, was observed more frequently than calcinosis in intermuscular fascial planes (29: 7). Subcutaneous calcinosis was demonstrated most commonly about the knees and elbows and in the fingers and toes. Intermuscular calcinosis affected the fascial planes around the quadriceps, deltoid, biceps, and the gastrocnemius/soleus muscles.Calcinosis, predominantly of the subcutaneous lesions, regressed spontaneously in eight of 11 children where this could be assessed over a one to four year period. This high rate of spontaneous resolution must complicate evaluation of the efficacy of proposed anti-calcinotic treatments in juvenile dermatomyositis.While no apparent relationship was established between subcutaneous calcinosis, which was present in all 29 of the affected patients, and disease activity or severity, all seven children with the additional, extensive, and 'classical' intermuscular fascial plane calcification developed this complication early and had severe disease.These findings are discussed in relation to previous studies.

A Giant Calcifying Pseudocyst Seen in a Patient with Overlap Syndrome

“…In connective tissue diseases, calcification is most frequently seen in dermatomyositis, and less frequently in systemic lupus erythematosus (4). Patients with dermatomyositis often show large lumps of calcification in their proximal limbs, muscles, and near large joints (1,2). In scleroderma, small calcium deposits appear in the subcutaneous tissue of the finger (3).…”

“…Some of these are seen in patients with various underlying diseases such as renal disorders, connective tissue diseases, and abnormal calcium metabolism. Subcutaneous calcifications in patients with, various types of connective tissue disease are type-specific, and case reports of calcification in patients with dermatomyositis (1,2), scleroderma (3), and systemic lupus erythematosus (4) have been published. In this paper we report a case of a female with overlap syndrome showing subcutaneous calcification.…”

Drug treatment of juvenile dermatomyositis.

“…Group 1 were those referred to us in the acute phase before drug treatment was started and managed exclusively by us (Table 1), and group 2 were those already established on drug treatment before referral ( Table 2). Patients in group 2 were subdivided further according to whether they had been referred during the acute phase of the illness (Cases 12-15, Table 2) or later (Cases [16][17][18][19][20][21][22][23][24][25][26][27][28][29].…”