Calcinosis cutis is common in several connective tissue diseases but rare in systemic lupus erythematosus (SLE). A 43-year-old woman with a 12-year history of SLE who presented with calcinosis cutis at the time of SLE diagnosis developed a large, ulcerated, draining mass on her left hip. The lesion was excised and skin was grafted, with an excellent early result. The clinical variants and mechanisms of ectopic calcification are discussed, as well as the proposed medical therapies for calcinosis cutis.Skin and subcutaneous tissue calcification, or calcinosis cutis, has been well documented in association with connective tissue diseases such as dermatomyositis and scleroderma, but rarely in association with systemic lupus erythematosus (SLE). We present a case of calcinosis cutis universalis in a patient with SLE, and we examine the role of surgical intervention as palliative therapy.
CASE REPORTA 43-year-old Mexican-American woman with a longstanding history of hypertension, non-insulindependent diabetes mellitus, and type IV hyperlipidemia was diagnosed at the age of 31 with SLE when she presented with malar rash, myalgias, arthralgias, and Raynaud's phenomenon. Several rock-hard, pea-sized, subcutaneous nodules were noted in her axillae and on her thighs. Submitted for publication July 8, 1996; accepted in revised form October 4, 1996. formed at that time, but a subsequent biopsy of a hard, ulcerated subcutaneous nodule showed calcinosis cutis.Gradually, the nodules increased in size and number and eventually coalesced, most impressively over the iliac crest regions, where they formed large, shield-like masses. Nodules also enlarged in the axillae and flexor surfaces of her forearms, the lower abdomen, groin, perianal region, thighs, and the popliteal fossae.Therapy included prednisone, colchicine, and hydroxychloroquine but was ineffective. Ulceration, infection, and abscess formation, with extrusion of chalky material from within the areas of calcification, was a recurring problem. Several histopathologic specimens showed calcinosis cutis.Her serum calcium and phosphate levels ranged between 8.3 and 9.7 mddl and 3.2 and 5.0 mg/dl, respectively. Creatine kinase was not elevated. Serum albumin concentrations ranged between 1.6 and 3.4 mg/dl. Serum parathyroid hormone and creatinine levels were also repeatedly normal. She had intermittent mild elevations in alanine transaminase, aspartate transaminase, and alkaline phosphatase levels, which occurred during symptomatic flares of SLE. Multiple studies were consistent with the diagnosis of SLE, including antinuclear antibody titers as high as 1:1,280, DNA antibodies 3-6 times normal, SS-NSS-B antibodies, thyroid microsoma1 antibodies, and markedly depressed levels of complement components C3 and C4. Radiographs of the extremities and pelvis and truncal computed tomography studies (Figure 1) showed extensive subcutaneous calcifications.The patient was referred for evaluation of a painful, ulcerated, shield-like area of calcinosis cutis over the left iliac crest t...