Abstract

(1) Background: Calcinosis cutis is a frequent symptom of autoimmune connective tissue diseases leading to pain, transcutaneous expulsion of calcified material and bacterial superinfection. There is a high need for new therapeutic options as no standardized treatment algorithm is established. While case reports indicate beneficial effects of bisphosphonates, standardized evaluation of treatment effects is missing. (2) Methods: In this retrospective analysis we evaluate the effects of intravenous pamidronate, a second-generation bisphosphonate, in seven patients with calcinosis cutis using consecutive clinical pictures, radiological examinations and patient’s subjective evaluation. (3) Results: 5/6 patients reported a reduction of pain, improvement of general condition and cessation of calcinosis progression. Regression of skin lesions was detectable in clinical pictures of 2/6 patients, while 1/6 patients had stable disease. Radiological examination revealed improvement or stable disease in 3/5 patients. Fever was the most common side effect. One out of seven patients developed osteonecrosis of the jaw. (4) Conclusions: Bisphosphonates appear to have beneficial effects in a subgroup of calcinosis cutis patients. While patient’s subjective evaluation was mainly positive, objective assessments showed improvement in approximately half of the cases. With regard to potential severe side effects, a careful risk-benefit evaluation is necessary before treatment initiation.

Highlights

  • Calcinosis cutis (CC) is a frequent symptom of autoimmune connective tissue diseases, which severely impacts patient’s quality of life

  • CC can occur in all autoimmune connective tissue diseases, but is most frequently seen in patients suffering from dermatomyositis and systemic sclerosis [2]

  • A case series of six patients showed no beneficial effects of etidronate on clinical and radiological assessments of CC in six patients with dermatomyositis and systemic sclerosis [8]. In this retrospective study we evaluate the effects of intravenous pamidronate in seven patients with dermatomyositis, systemic sclerosis or mixed connective tissue diseases

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Summary

Introduction

Calcinosis cutis (CC) is a frequent symptom of autoimmune connective tissue diseases, which severely impacts patient’s quality of life. It is defined as a deposition of insoluble calcium in the subcutaneous tissue, while serum calcium and/or phosphate levels are not elevated in most cases [1]. CC forms subcutaneous nodules, which are usually localized at mechanically stressed body sites, such as fingers or above joints. Often it causes inflammation and devastating perforations of the skin, leading to pain and baring the risk of bacterial superinfection. Published treatment attempts include intralesional corticosteroid injections, topical or intravenous thiosulfate, warfarin, minocycline, colchicine, calcium channel blockers, intravenous im-

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