Surgical Debulking for Symptomatic Management of Calcinosis Cutis of the Hand and Upper Extremity in Systemic Sclerosis

Author(s):  
Kevin M. Klifto ◽  
Brian H. Cho ◽  
Scott D. Lifchez
2021 ◽  
pp. 239719832110043
Author(s):  
Miguel Angel Garcés Villalá ◽  
Carolina Zorrilla Albert

Introduction: Limited cutaneous systemic sclerosis with special manifestations (calcinosis cutis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) is part of the group of connective tissue diseases, these rare autoimmune systemic pathologies cause thickening and hardening of tissues in different parts of the body and can lead to complex disorders. Oral manifestations of systemic sclerosis may include limited ability to open the mouth, xerostomia, periodontal disease, enlarged periodontal ligament, and bone resorption of the jaw. Case Description: A 54-year-old Caucasian patient presented with oral pain, swallowing, phonation and chewing difficulties associated with dental instability, hygiene/handling difficulties and her main problem with microstomia, which prevented her from removing the skeletal prosthesis for 4 years, depriving her of social life. Gradual treatment with dental implants was diagnosed and planned to support a fixed total denture adapted to the ridge with self-cleaning characteristics. After implant insertion, panoramic radiographs with standardized parameters were taken to compare crestal bone levels at the time of prosthesis placement and with 10 years of follow-up. Conclusion: The average crestal bone loss of the 12 implants after the 10 years of follow-up was 1.26 mm for the maxilla and 1.17 mm for the mandible. The survival of the 12 support implants of two total fixed prostheses in a clinical/radiographic follow-up of 10 years was 100%. After 10 years of follow-up, the 12 implants inserted had a bone loss similar to that of healthy patients and no pathologies were registered, recovering function, aesthetics, and self-esteem. This therapy must be implemented before the interincisal distance decreases to 30 mm to allow intraoral surgical/prosthetic access. Implant-supported total fixed rehabilitation is a viable, predictable, and recommended therapy in patients with limited cutaneous systemic sclerosis.


2020 ◽  
Vol 12 (6) ◽  
pp. 127-128
Author(s):  
Susana Rodrigues ◽  
Catrine Ferreira ◽  
Tiago Coelho ◽  
Diogo Gaspar ◽  
Jean Fallah ◽  
...  

Calcinosis cutis is a rare disorder characterized by of deposition of insoluble calcium salts in the skin and subcutaneous tissue. Five subtypes of calcinosis cutis are described: dystrophic, metastatic, idiopathic, iatrogenic, and calciphylaxis.1 Of these subtypes, dystrophic calcinosis (DC) is the most common, and it is the most frequently seen in association with underlying autoimmune connective tissue diseases.2 Dermatomyositis, systemic sclerosis and less commonly systemic lupus erythematous were described to be complicated by DC. However, DC associated with rheumatoid arthritis (RA) is extremely rare.2 The condition causes substantial morbidity and is associated with pain and limitation of movement when the process involves areas close to joints or when ulceration occurs.2 We report a middle age Sudanese woman with good controlled RA who developed dystrophic calcinosis cutis.


2020 ◽  
Vol 7 (8) ◽  
pp. 2803
Author(s):  
Surya Rao Rao Venkata Mahipathy ◽  
Alagar Raja Durairaj ◽  
Narayanamurthy Sundaramurthy ◽  
Anand Prasath Jayachandiran

Calcinosis cutis is abnormal calcium deposition in the skin and subcutaneous tissues of the body. It is generally associated with autoimmune connective tissue disorders and in our case, it is systemic sclerosis. It most commonly occurs in the fingers presenting with pain and functional impairment. Here, we present a case of calcinosis cutis with systemic sclerosis in a teenage girl presented with bilateral gluteal pressure sores and multiple sites of calcium deposition like sacrum, upper limbs, knees and the face. We treated here with reconstructive surgery with Limberg flaps for the pressure ulcers with excision and primary closure of the other sites with calcium deposits. 


2019 ◽  
Vol 44 (11) ◽  
pp. 990.e1-990.e7 ◽  
Author(s):  
Jacinta Leyden ◽  
Matthew B. Burn ◽  
Victor Wong ◽  
Daniel Sotelo Leon ◽  
Yukitoshi Kaizawa ◽  
...  

2015 ◽  
Vol 41 (1) ◽  
pp. 94-96 ◽  
Author(s):  
H. Poormoghim ◽  
E. Andalib ◽  
A. R. Almasi ◽  
E. Hadibigi

QJM ◽  
2021 ◽  
Author(s):  
Sumantro Mondal ◽  
Debanjali Sinha ◽  
Alakendu Ghosh

Abstract Soft tissue calcification is seen in some rheumatological diseases, including systemic sclerosis. We herein present a clinical image of calcinosis cutis of finger pulps and its characteristic radiographic image in a patient with limited cutaneous systemic sclerosis.


2021 ◽  
pp. 239719832110532
Author(s):  
Antonia Valenzuela ◽  
Lorinda Chung

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.


2020 ◽  
Vol 74 (4_Supplement_1) ◽  
pp. 7411520489p1
Author(s):  
Susan Murphy ◽  
Donnamarie Krause ◽  
Erin Welsh ◽  
Angela Lyden ◽  
Mary Barber ◽  
...  

2018 ◽  
Vol 4 (2) ◽  
pp. 149-159
Author(s):  
Yannick Allanore ◽  
Farhad Gharibdoost ◽  
Ahmad Reza Jamshidi ◽  
Ali Javinani ◽  
Jérôme Avouac ◽  
...  

Objectives: Systemic sclerosis is a severe and rare chronic auto-immune multisystem disorder characterized by vasculopathy and skin stiffness. Ethnic and geographical origin can influence the outcomes. In this study, we compared the phenotypic characteristics of Iranian and French patients. Methods: This cross-sectional study was performed on 200 Iranian and 268 French systemic sclerosis patients. Iranian patients collected from the Iranian systemic sclerosis cohort of the Rheumatology Research Center, Shariati hospital, Tehran University of Medical Sciences. The French population was monocentric, and it was constituted by the patients included locally in the EUSTAR database in December 2016. Results: The mean age at onset was significantly lower in Iranian patients (35.58 ± 11.68 vs 47.06 ± 13.54, p-value < 0.001). The female-to-male ratio was approximately 5.2:1 and was not different in the two populations. The prevalence of diffuse cutaneous systemic sclerosis was significantly higher in Iranian patients (60.2% vs 42.85%, p-value < 0.001). Calcinosis cutis and joint synovitis were more prevalent in French patients ( p-value = 0.013, <0.001). The positivity of anti-topoisomerase antibody was higher in Iranian patients, whereas the anti-centromere antibody predominated in French cases ( p-value < 0.001). Restrictive pattern of pulmonary function test was more common in Iranian patients ( p-value < 0.001), while estimated pulmonary arterial pressure by echocardiography was higher in French patients ( p-value < 0.001). Conclusion: It seems that systemic sclerosis occurred in younger ages among Iranian female with the predominance of diffuse cutaneous subtype. In addition, lung interstitial disease appeared to be more prevalent and severe in Iranians than French patients.


2020 ◽  
Vol 82 (2) ◽  
pp. 317-325 ◽  
Author(s):  
Hélène Traineau ◽  
Rohit Aggarwal ◽  
Jean-Benoît Monfort ◽  
Patricia Senet ◽  
Chester V. Oddis ◽  
...  

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