Idiopathic calcinosis cutis of the right iliac region: Common lesion at uncommon

Calcinosis cutis is an uncommon soft tissue lesion characterized by the deposition of calcium salts in the skin or subcutaneous tissue attributed to a wide variety of causes. We present a case of idiopathic calcinosis cutis in an adult male, who presented with a swelling in the right iliac region. Chalky white aspirate and amorphous basophilic granular material on microscopy suggestive of calcium deposits were noted. Histopathological examination of the excised mass coupled with appropriate clinical background led to the final diagnosis of idiopathic calcinosis cutis. We present this case with a complete diagnostic workup to undermine the importance of considering this lesion in the differential diagnoses of a subcutaneous hard lump in an otherwise healthy patient.

Rituximab and intravenous immunoglobulin treatment in PM/Scl antibody-associated disease: case-based review

Autoantibodies to the 75-kDa and 100-kDa subunits of the PM/Scl nucleolar protein complex are associated with an overlap syndrome, manifesting with clinical features of systemic sclerosis and idiopathic inflammatory myopathy. We describe the diverse clinical features in a series of 4 cases with anti-PM/Scl-75 and/or anti-PM/Scl-100 antibodies, including severe proximal muscle weakness, oesophageal dysfunction, respiratory weakness requiring mechanical ventilation, Raynaud’s, calcinosis cutis, sclerodactyly and critical digital ischaemia. Despite the severity of striated and oesophageal muscle weakness, all patients responded very well to immune suppression, and calcinosis cutis in one case regressed substantially. We highlight the efficacy of Rituximab and intravenous immunoglobulin therapy (IVIg) in these cases, enabling return to normal muscle function within six months. Rituximab was preferentially chosen for cases with hyper-gammaglobulinemia and multiple autoantibodies in addition to anti-PM/Scl, and IVIg was utilised for cases where a rapid onset of effect was required, such as severe ventilator-dependent respiratory muscle weakness and oesophageal dysfunction.

Bisphosphonates for the Treatment of Calcinosis Cutis—A Retrospective Single-Center Study

(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.

A rare case report of neonatal calcinosis cutis induced by distant and delayed extravasation of intravenous calcium gluconate

A 3,480 g male neonate showed tachypnea symptom with a serum ionized calcium level of 0.66 mmol/L by routine clinical analysis. He was injected calcium gluconate intravenously through femoral vein catheter to treat the hypocalcemia. On second day after the injection, he started to show erythema in the flank area. The lesion became firm and changed into whitish crust consist of small crystals. Abdominal X-ray and ultrasonography showed the accumulation of calcium deposit in the subcutaneous layer of the lesion. Surgical debridement was performed to remove the crust with calcium deposit and acellular fish skin graft rich in omega-3 (Kerecis) was applied to the defect site for secondary intention of the defect wound. After 2 months, the skin and soft tissue defect were fully covered with healthy normal skin without depression or contracture. This report is a first case of iatrogenic calcinosis cutis without extravasation symptom.

Subcutaneous calcinosis: Is it different between systemic sclerosis and dermatomyositis?

Calcinosis cutis is the deposition of insoluble calcium in the skin and subcutaneous tissues. It is a manifestation of several autoimmune connective tissue diseases, most frequently with systemic sclerosis and juvenile dermatomyositis, followed by adult dermatomyositis. Autoimmune connective tissue disease–associated calcinosis is of the dystrophic subtype, which occurs at sites of damaged tissue in the setting of normal serum calcium and phosphate levels. In juvenile dermatomyositis, calcinosis is considered a marker of ongoing disease activity and possibly inadequate treatment, while in adult dermatomyositis, it is a hallmark of skin damage due to chronic rather than active disease. Calcinosis is associated with long disease duration in systemic sclerosis and dermatomyositis, anti-polymyositis/sclerosis autoantibodies in systemic sclerosis and NXP-2 and melanoma differentiation-associated gene 5 in dermatomyositis. Calcinosis in systemic sclerosis occurs most frequently in the hands, particularly the fingers, whereas in dermatomyositis, it affects mainly the trunk and extremities. The primary mineral component of calcinosis is hydroxyapatite in systemic sclerosis and carbonate apatite in dermatomyositis. Calcinosis in dermatomyositis and systemic sclerosis share some pathogenic mechanisms, but vascular hypoxia seems to play a more important role in systemic sclerosis, whereas the release of calcium from mitochondria in muscle cells damaged by myopathy may be a primary mechanism contributing to dermatomyositis-related calcinosis. Multiple treatment strategies for dermatomyositis and systemic sclerosis-related calcinosis have been used with variable results. Early aggressive treatment of underlying myositis in patients with dermatomyositis may improve long-term outcomes of calcinosis. A better understanding of the pathogenesis of calcinosis is needed to improve treatment options.