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Calcinosis Cutis
Published in Charles Theisler, Adjuvant Medical Care, 2023
Calcinosis cutis is a condition where calcium salt crystals accumulate within the dermis (skin). Lesions usually appear as small, firm, white or yellow lumps (papules, plaques, or nodules) on the surface of the skin that may be hard or soft. A solitary lesion may develop, although multiple lesions are more common. Lesions may become tender and ulcerate, discharging a creamy chalk-like material consisting mainly of calcium phosphate with a small amount of calcium carbonate.1 Calcinosis usually has no symptoms but can be painful in some cases. Calcinosis cutis commonly occurs in patients with systemic sclerosis and dermatomyositis. There are four subtypes of this disorder. Any underlying cause (e.g., hypercalcemia and/or hyperphosphatemia) should be corrected.
Infection in the Hematopoeitic Stem Cell Transplant Recipient with Autoimmune Disease
Published in Richard K. Burt, Alberto M. Marmont, Stem Cell Therapy for Autoimmune Disease, 2019
Valentina Stosor, Teresa R. Zembower
Skin loses elasticity and is easily broken and infected at points of contact. Esophageal dysfunction can lead to aspiration with pneumonia, and reduced gut motility often leads to bacterial overgrowth syndromes. Calcinosis can lead to skin ulcerations that become secondarily infected, occasionally resulting in soft tissue infections and gangrene.16,17 One case of pyomyositis in a patient with progressive SSc has been reported. Interstitial lung disease and pulmonary fibrosis can mimic pneumonia and complicate the diagnosis of infection in these patients.16,17
Rheumatology
Published in Stephan Strobel, Lewis Spitz, Stephen D. Marks, Great Ormond Street Handbook of Paediatrics, 2019
Clarissa Pilkington, Kiran Nistala, Helen Lachman, Paul Brogan
Erythematous nail-beds with dilated capillary loops are often seen; a generalised vasculopathy occurs in severe disease, with skin ulceration, gut involvement and CNS involvement. Calcinosis may be present at onset, or occurs later if disease is not controlled.
Prevalence and clinical association with calcinosis cutis in early systemic sclerosis
Published in Modern Rheumatology, 2021
Chawiporn Muktabhant, Punthip Thammaroj, Prathana Chowchuen, Chingching Foocharoen
Calcinosis cutis is the deposition of insoluble calcium in the skin and subcutaneous tissues. The prevalence of calcinosis cutis in SSc ranges from 18 to 49% [5,6]. Calcinosis cutis occurs most frequently in the hands, followed by proximal upper extremities, knees or proximal lower extremities and hip, in order [5]. The pathophysiology of calcinosis cutis remains poorly understood. Chronic inflammation and vascular hypoxia are thought to play a role in the tissue damage that serves as a nidus for dystrophic calcification [7]. The other mechanisms include recurrent trauma and abnormalities in bone matrix proteins [5]. Common and potentially debilitating complications of calcinosis cutis include pain, local inflammation, ulceration, and infection [7]. Although diagnosis of calcinosis cutis is primarily performed by physical examination, radioimaging can help detection of subclinical deposits. Plain radiography is quite sensitive in detecting calcinosis cutis and is recommended as an initial imaging evaluation of calcinosis cutis [5,7].
Systemic sclerosis-related calcinosis can affect the breast – but malignancy should always be excluded
Published in Scandinavian Journal of Rheumatology, 2021
EY Tang, S Varughese, AL Herrick
There is currently no effective disease-modifying treatment for calcinosis (2). Despite the background of widespread calcinosis in this patient and the probability that the breast lump was due to SSc-related calcinosis, she received a comprehensive assessment to ensure that the lesion was non-malignant. To date, SSc-related breast calcinosis is not well recognized in the literature. Our case demonstrates how it is important to consider calcinosis in the differential diagnosis of breast lesions in patients with SSc, but nonetheless any new lesion must always be actively investigated to exclude other causes such as malignancy. In our patient, given the diagnosis of SSc, her background of calcinosis, and the findings of the investigations, it was possible to provide reassurance and to avoid unnecessary surgery.
Inflammatory myopathies: shedding light on promising agents and combination therapies in clinical trials
Published in Expert Opinion on Investigational Drugs, 2021
Rachel Zeng, Stefanie Glaubitz, Jens Schmidt
Calcinosis involves the deposition of carbonate apatite in soft tissue and is a severe and painful complication in autoimmune connective tissues diseases; in myositis syndromes it most commonly affects patients with DM or OM [3]. Various treatments of calcinosis have been proposed, but evidence is limited to case reports or retrospective case series. In a single-center retrospective cohort study of 16 myositis patients affected by calcinosis (11 DM, 4 OM, 1 PM), different calcinosis-specific treatments included diltiazem, bisphosphonates, warfarin, colchicine, IVIg, rituximab, infliximab, and sodium thiosulfate ointment, but none of these treatments showed sufficient effects on the reduction of calcinosis or the prevention of new sites involved [73]. Another single-center cohort study with 35 adult DM patients with calcinosis also showed no difference in treatment response between the bisphosphonate treatment group and the group not receiving bisphosphonate [74]. Regarding the use of rituximab, some case reports suggested beneficial effects, while others showed no improvement of calcinosis (summarized in [75]). There are recent reports on the successful use tofacitinib [76] or TNFα inhibitor [77] in myositis-associated calcinosis. In a retrospective case series involving the use of topical sodium thiosulfate in 28 patients with calcinosis cutis, 19 (68%) showed clinical improvement [78]. Currently, a prospective open-label study for the evaluation of efficacy and safety of intravenous sodium thiosulfate in DM patients with moderate to severe extensive calcinosis is ongoing [ClinicalTrials.gov Identifier: NCT03267277].