October 11, 2016

Calcinosis: An Intractable and Distressing Problem (Slideshow)

Better treatment urgently needed

Dystrophic calcinosis is the most common form of calcinosis, the deposition of insoluble calcium salts in various tissues. It occurs most frequently in patients with underlying autoimmune rheumatic diseases such as scleroderma and dermatomyositis.

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These slides depict calcinosis in several patients, including a 29-year-old female with juvenile dermatomyositis since age 7 whose myositis has remained in remission on prednisone, hydroxychloroquine and intravenous immunoglobulin (IVIG) infusions. However, her subcutaneous calcium deposits progressed relentlessly, ultimately covering her entire body like an exoskeleton.

These cases demonstrate the devastating nature of dystrophic calcinosis and the urgent need for further research to develop effective therapies.

<p>Calcinosis is a particular problem in limited scleroderma (formerly CREST syndrome – calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia). Calcinosis most frequently forms at sites of repeated microtrauma, including the hands (particularly fingers) and feet, followed by more proximal extremities.</p>

<p>Dystrophic calcinosis occurs in dermatomyositis and is more common in the juvenile form than in this classic (adult) form. This radiograph shows a 78-year-old female with dermatomyositis and extensive calcification of lobular morphology projecting over the majority of the pelvis.</p>

<p>Plaques and exophytic growths significantly restrict this patient’s mobility, making intravenous access and even urination and defecation extremely difficult.</p>

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<p>Patients present with skin-colored, white, yellow or erythematous firm papules, nodules or plaques that may ulcerate and extrude chalky white material.</p>

<p>Dystrophic calcinosis tends to develop over extremities, primarily elbows, knees and shoulders, and above the buttocks. Deposits may be intracutaneous, subcutaneous, fascial or intramuscular and can be painful. This is the elbow of a patient with juvenile dermatomyositis.</p>

<p>The extent of calcinosis can be ascertained on radiographs of the affected parts of the body. Right elbow and right forearm radiographs show extensive subcutaneous and intramuscular/fascial calcification with a large area of calcification in the olecranon bursa. The calcification involves all muscle groups.</p>

<p>The calcinotic mass from the patient’s right elbow (pictured in slide 5) ruptured and partially extruded through her skin, necessitating surgical excision to temporarily alleviate the problem.</p>

<p>Even surgical excision in this patient had no lasting benefit as her calcinosis gradually returned at the surgical site over the next year.</p>

<p>The deep, intramuscular or fascial forms of dystrophic calcinosis may limit muscle contraction and joint motion. This radiograph of a 76-year-old female with scleroderma shows extensive soft tissue calcification in the upper extremity predominant on the dorsal aspect of the forearm and distal arm as well as around the dorsal aspect of the elbow.</p>

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Images and text provided by Soumya Chatterjee, MD, Director, Scleroderma Program, Department of Rheumatic and Immunologic Diseases.

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