Calcinosis cutis in a young man with dermatomyositis

Abstract

A 25-year-old man with known dermatomyositis was admitted to the University of Leipzig Medical Centre for the first time. The diagnosis was originally made abroad years ago. Current laboratory studies showed elevated C-reactive protein and creatine kinase. Antinuclear antibodies, anti-Mi-2 antibodies, anti-Jo-1 antibodies, and anti-Scl-70 antibodies were negative, and the rheumatoid factor was positive. Nailfold video capillaroscopy (NVC) revealed numerous arborized (or “bushy”) capillaries reflecting dermatomyositis-typical neoangiogenesis. Physical examination showed extensive calcinosis cutis on arms, legs, and trunk. Impressively, we found numerous painful nodules and subsequent scars all over his body (exemplarily demonstrated on the right arm, Figure 1A). He reported these lesions were repeatedly associated with surrounding skin inflammation. Additional complaints were generalized myalgia and symmetrical proximal muscle weakness of the upper legs. X-ray of the affected right arm shows multiple nodular, partly confluent calcifications affecting the cutis and the subcutis (Figure 1B). The only treatment for the patient's dermatomyositis was daily administration of prednisolone up to 50 mg for several years. For steroid-sparing reasons, we initiated an immunosuppressive treatment with azathioprine (125 mg daily). The prednisolone dose could be reduced to 2.5 mg hereafter. To treat the calcinosis cutis, minocycline was prescribed.1, 2 Additionally, iloprost was administered to improve a secondary Raynaud's phenomenon. Unfortunately, none of these treatment approaches led to a relevant improvement of the calcinosis cutis. In general, calcinosis cutis seems to be more common in juvenile dermatomyositis than in adult patients.3, 4 Our patient was diagnosed during adolescence. Calcinosis cutis, in the context of rheumatic diseases, is usually dystrophic and characterized by tissue deposition of calcified material without elevated serum calcium or phosphate levels. The exact causes are not known, but recurrent trauma, vascular hypoxemia, and chronic inflammation are theorized to be involved.2 Pharmacologic treatment options are limited and data is scarce. Possible pharmacotherapies include minocycline, diltiazem, bisphosphonates, colchicine, the anti-CD20 antibody rituximab, or intravenous immunoglobulins. Surgical excision of the lesions is possible but should be restricted to selected (i.e., large or particularly painful) lesions since impaired wound healing and infections are not uncommon.2 Marco Krasselt: Conceptualization; data curation; project administration; resources; supervision; visualization; writing and review of the original draft. Jeanette Henkelmann: Data curation; validation; visualization; writing and review of the original draft. Matthias Pierer: Data curation; resources; validation; writing and review of the original draft. The authors have nothing to report. The authors declare no conflict of interest. The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and any other clinical information to be reported in the article. The patient understands that his name and initial will not be published and due efforts will be made to conceal the identity of the patient, although anonymity cannot be guaranteed. The information will be published without the patient's personal information and every attempt will be made to ensure anonymity. Some or all data of this article are available from the corresponding author on reasonable request.