Hammad Ali1, Connor R Buechler2,3, Kristine M Lohr1. 1. Division of Rheumatology, Department of Medicine, University of Kentucky, Lexington, KY, USA. 2. Department of Medicine, University of Minnesota, Minneapolis, MN, USA. 3. Department of Dermatology, University of Minnesota, Minneapolis, MN, USA.
A 57-year-old woman with oesophageal dysmotility and complaint of ‘crystals’ emerging from wounds presented to the clinic. She brought crystals (Fig. 1) that had grown and fallen from the wounds. Examination was notable for sclerodactyly distal to the wrists, in addition to ulcerated, painful nodules extruding a whitish-yellow chalk-like matter on her bilateral shins (Fig. 1) and the ulnar border of the left forearm. Blood work revealed ANA 1:640 (centromere pattern), positive anti-centromere antibody and negative anti-RNA polymerase III, anti-Scl-70, anti-Smith, anti-dsDNA, anti-SSA, anti-SSB and anti-RNP. Serum calcium, phosphorus, vitamin D and parathyroid hormone were normal. A diagnosis of limited SSc with advanced calcinosis cutis was made. Treatment was initiated with MMF 1.5 g twice daily, diltiazem 120 mg twice daily and colchicine 0.6 mg daily. After 6 months without improvement of the calcinosis, the colchicine was stopped, and minocycline was started at 100 mg daily with follow-up in 6 months. Treatment for calcinosis cutis remains challenging, especially in advanced disease. Intralesional sodium thiosulphate, IVIG, minocycline, colchicine, CYC and rituximab have shown some promise; however, there is a dearth of large evidence-based studies [1]. Surgical excision can provide cosmetic benefits, but it does not prevent recurrence [2]. Treatment of the underlying disease early and aggressively to prevent morbidity is the current consensus.
Figure 1.
Calcinosis cutis of left shin with patient's collection of calcium crystals that had fallen out
Calcinosis cutis of left shin with patient's collection of calcium crystals that had fallen out