Calcinosis Cutis and Calciphylaxis in Autoimmune Connective Tissue Diseases

Abstract

Calcinosis represents a severe complication of several autoimmune disorders. Soft-tissue calcifications have been classified into five major types: dystrophic, metastatic, idiopathic, iatrogenic, and calciphylaxis. Autoimmune diseases are usually associated with dystrophic calcifications, including calcinosis cutis, occurring in damaged or devitalized tissues in the presence of normal serum levels of calcium and phosphate. In particular, calcinosis cutis has been described in dermatomyositis, polymyositis, juvenile dermatomyositis, systemic sclerosis, systemic lupus erythematosus, primary Sjögren's syndrome, overlap syndrome, mixed connective tissue disease, and rheumatoid arthritis. Calciphylaxis, a severe and life-threatening syndrome presenting with vascular calcifications and thrombosis, has also been associated with some autoimmune conditions. Due to the potentially disabling character of calcinosis cutis and calciphylaxis, physicians' awareness about the clinical presentation and management of these diseases should be increased to select the most appropriate treatment option and avoid long-term complications. In this review, we aim to analyze the clinical features of calcinosis cutis and calciphylaxis associated with autoimmune diseases, and the main treatment strategies evaluated up to now for treating this potentially disabling disease.

Keywords: autoimmune diseases; calcinosis cutis; comorbidity; connective tissue diseases; rheumatoid arthritis.

Figure 1

Cutaneous calcinosis in systemic sclerosis (SSc). Cutaneous calcinosis of the right superior limb complicated with ulceration in a female patient with morphea (A). Calcinotic masse of the right inferior limb in a male patient with SSc, complicated with two ulcerative lesions (B).

Figure 2

Cutaneous calcinosis in dermatomyositis (DM). Multiple and extensive calcinosis cutis (arrowheads) of the right inferior limb complicated with ulceration in a female patient with DM. Fibrinous exudate (asterisk) is present in the cavity of the wounds. The arrow indicates residues of antiseptic cream.

Figure 3

Cutaneous calcinosis in rheumatoid arthritis (RA). Calcinosis cutis in the soft tissue at the tip of the finger (arrow) in a female patient with RA (A). Multiple calcinotic lesions of the left leg (arrows) complicated with necrotizing ulcers in a female patient with RA. The asterisk indicates a concomitant chronic venous ulcer (B).