Treatment Of Calcinosis Cutis Research Articles

P28 Recalcitrant exuberant digital calcinosis cutis in a patient of CREST syndrome - A case report

Introduction/BackgroundCalcinosis cutis is dystrophic soft-tissue calcification associated with connective tissues diseases, especially scleroderma, systemic lupus erythematosus or dermatomyositis. It occurs in all subsets of scleroderma but is more prominent in patients with limited scleroderma (CREST syndrome) and in those with anticentromere antibody. Dystrophic calcinosis can be associated with severe pain, decreased mobility, increased risk of infection, and significantly decreased quality of life. We report our experience with a case of refractory calcinosis cutis in a patient with multiple comorbidities.Description/MethodWe are presenting a 62-year-old lady diagnosed with Limited Cutaneous Sclerosis (CREST syndrome) with background of Giant Cell Arteritis, Osteoporosis, TIA and Hypothyroidism. Patient has had a history of onset of Raynaud’s at the age of 20 years. Around the age of 50, she started developing gastrointestinal features and subcutaneous nodules on her fingers. The painful nodules on her fingers were her predominant symptom. These nodules appeared as multiple hardened exuberant erythematous-whitish nodules, some having a chalky appearance, around her fingers. A clinical diagnosis of calcinosis cutis was made. Other features included sclerotic cutaneous findings on her face and fingers (sclerodactyly), prominent facial telangiectasia and esophageal reflux. In light of suspected CREST syndrome, patient was extensively investigated, and diagnosis was confirmed. Antinuclear and anti-centromere (anti-Th and anti-RNP) antibodies were positive. X-ray showed extensive calcinosis in the soft tissue of the fingers. Serum calcium and phosphorus levels were within the normal ranges. Pulmonary function test was normal. For her ongoing digital calcinosis cutis associated with severe pain and recurrent ulceration which was adversely impacting the quality of her life, she was started on low dose aspirin to alleviate her symptoms. This initially helped improve her symptoms, however later stopped responding. Then a trial of low dose Irbesartan at dose of 75 mg was given with which she found no relief. She was referred to higher center and a trail of Minocycline at dose of 50-100mg was given for over 3 months which proved to be of no benefit. Reports suggest bisphosphonates are helpful in Calcinosis cutis and though patient was Zoledronic acid for osteoporosis, her calcinosis cutis continued to grow and ulcerate. In view of recalcitrant nature of these lesions, patient has been referred to the plastic surgeons for surgical modality of treatment currently.Discussion/ResultsCalcinosis cutis is a rare and chronic condition characterized by deposition of insoluble calcium salts in the skin and subcutaneous tissues. Dystrophic calcinosis is the most common type of calcinosis cutis and is seen in association with autoimmune connective tissue diseases. It is thought to occur as a result of chronic local tissue injury and is a common complication of systemic sclerosis especially the limited form (CREST syndrome: calcinosis, raynaud's phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia), affecting approximately 25% of these patients. Calcinosis may develop at any time during the disease course and may predate the diagnosis of scleroderma, but typically occurs at least 10 years following diagnosis. Lesions occur on the hands and feet, with a high predilection for fingertips and areas of microtrauma. Raynaud’s and digital ulcers are risk factors for calcinosis in scleroderma, suggesting a role for vascular ischemia. These lesions might be associated with pain, soft tissue swelling, ulcers with toothpaste-like material discharging or deformities, which may lead to functional problems. Treatment of calcinosis cutis is tough and challenging as there is no gold standard treatment. Medical therapy for cutaneous calcinosis is limited and has variable benefits. Multiple treatment approaches with diltiazem, disodium etidronate, probenecid, colchicine, minocycline, low-dose warfarin, intralesional adrenal steroids, abatacept, rituximab, thalidomide have been explored, but no standard treatment has convincingly prevented or reduced calcinosis. Surgery is generally reserved for discrete lesions with problems of recurrent infection, severe pain, or functional impact Despite concerns about recurrence of lesion due to mechanical trauma at the operative site, surgical resection has been effective in treating calcinosis. Treatment with extracorporeal shock wave lithotripsy and carbon dioxide laser therapy have shown promise in a few cases and need further study.Key learning points/ConclusionPharmacological treatment of calcinosis cutis is difficult and a variety of drugs including bisphosphonates, intralesional corticosteroids, aluminium hydroxide, warfarin and diltiazem, have been tried with limited success. The local excision of painful or ulcerated nodule is the current existing therapeutic potion but local recurrence is common. The present case is of interest because it has features of Raynaud’s phenomenon, heart burn, telangiectasia and sclerodactyly with microscopic finding of calcinosis cutis (CREST syndrome). Patient with crest syndrome often have better prognosis than diffuse systemic sclerosis. Pathologist should be aware about various clinico-pathological categories associated with localized calcium deposition in dermis.

Calcinosis cutis in the auricle: a case report

Calcinosis cutis is a rare condition defined as the pathological accumulation of amorphous insoluble calcium salts in cutaneous and subcutaneous tissue. There are four subtypes - dystrophic, idiopathic, metastatic, and iatrogenic calcification. The dystrophic form is the most common type of calcinosis cutis, the major agent being trauma. Serum calcium and phosphorus values are within normal limits in these patients. The clinical manifestation generally involves small white papules, and subcutaneous nodules or plaques. The first choice in the treatment of calcinosis cutis is surgery. Surgical procedures have been recommended as the first and most effective option in almost all cases in the literature. We report a case of a patient presenting with growing, painless swelling following trauma to the auricle of the ear identified as dystrophic calcinosis cutis following surgical excision.

Calcinosis cutis in the auricle: a case report

Calcinosis cutis is a rare condition defined as the pathological accumulation of amorphous insoluble calcium salts in cutaneous and subcutaneous tissue. There are four subtypes - dystrophic, idiopathic, metastatic, and iatrogenic calcification. The dystrophic form is the most common type of calcinosis cutis, the major agent being trauma. Serum calcium and phosphorus values are within normal limits in these patients. The clinical manifestation generally involves small white papules, and subcutaneous nodules or plaques. The first choice in the treatment of calcinosis cutis is surgery. Surgical procedures have been recommended as the first and most effective option in almost all cases in the literature. We report a case of a patient presenting with growing, painless swelling following trauma to the auricle of the ear identified as dystrophic calcinosis cutis following surgical excision.

Кальциноз при системній склеродермії: поширеність, клініка, менеджмент, ускладнення

У даному літературному огляді підсумовано сучасні дані щодо епідеміології, патофізіології, діагностики та лікування шкірного кальцинозу у пацієнтів із системною склеродермією (ССД). Проведено аналіз обсерваційних досліджень, що описують частоту кальцинозу при ССД і асоційованих з ним клінічних ознак; молекулярних досліджень, що вивчають потенційні патогенетичні механізми; наведені клінічні випадки, що описують нові діагностичні підходи і методи лікування кальцинозу. Шкірний кальциноз — це відкладення нерозчинного кальцію в шкірі і підшкірних тканинах. Кальциноз — одна з основних, погано керованих клінічних проблем у пацієнтів із ССД, яка зачіпає щонайменше одну четверту частину хворих. Кальциноз асоційований з більшою тривалістю хвороби, дигітальними виразками, акроостеолізом, позитивними антицентромерними і антитопоізомеразними антитілами. Хоча патогенез кальцинозу остаточно невідомий, є докази, що підтверджують роль місцевої хронічної травматизації, хронічного запалення, судинної гіпоксії і дисрегуляції білків кісткового матриксу як потенційних механізмів розвитку цього патологічного стану у пацієнтів із ССД. Діагноз може бути встановлений на основі клінічних даних або за допомогою стандартної рентгенографії. Кілька фармакологічних методів лікування були застосовані у пацієнтів із кальцинозом зі змінними і не такими значущими результатами, у свою чергу, хірургічне видалення депозитів кальцію залишається основним методом лікування.

Topical sodium thiosulfate as treatment of calcinosis cutis: Case series and systematic review

To the Editor: Sodium thiosulfate (STS) and sodium metabisulfite (SMS), which forms a metabolite of STS in vivo, represent promising therapeutic options for calcinosis cutis (CC).1,2 We conducted a systematic review to summarize the published data on the efficacy and safety of topical STS/SMS as a therapy for CC. This review was registered on the PROSPERO database (CRD42019129818) and executed according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines.3 The literature search was performed using MEDLINE, Embase, SCOPUS, ScienceDirect, and PubMed, from each database's inception to October 16, 2020.

Treatment of calcinosis cutis with sodium thiosulfate therapy

To the Editor: Calcinosis cutis (CC) is a rare cutaneous condition defined by abnormal depositions of insoluble calcium salts in the skin and subcutaneous tissue.1 Finding effective and safe treatments for these calcified lesions has been a known challenge among dermatologists. A variety of therapies have been reported, with limited success, including the treatment of CC with the use of warfarin, bisphosphonates, minocycline, intralesional corticosteroids, surgical excision, and carbon dioxide laser therapy.

Epidemiology and Treatment of Calcinosis Cutis: 13 Years of Experience.

Background:Calcinosis cutis is a rare condition associated with different diseases, which is difficult to manage.Aims and Objectives:In this retrospective study, the epidemiology of calcinosis cutis and the effectiveness of various treatment regimens in its management were assessed in a single center.Materials and Methods:The data of 34 patients suffering from calcinosis cutis (male:female = 12:22; mean age = 48.6 ± 18.6 years) treated at our department between 2003 and 2016 were analyzed retrospectively.Results:Dystrophic, idiopathic, metastatic subtype, and calciphylaxis occurred in 70.6%, 11.8%, 5.9%, and 11.8% of the cases, respectively. Underlying diseases of dystrophic calcinosis included autoimmune connective tissue disease, skin trauma, cutaneous neoplasm, and inherited disorder in 58.3%, 20.8%, 12.5%, and 8.3% of the cases, respectively. Extremities were most frequently affected (n = 18). In the management, diltiazem was most frequently used in monotherapy with partial response in five of eight cases. Other drugs in monotherapy or in combination were administered in single cases. Surgical treatment resulted in least partial response in all of the cases followed (n = 7).Conclusion:Dystrophic was the most common subtype and autoimmune connective tissue disease was the most frequent underlying disease. We conclude that lower doses of diltiazem have only partial efficiency, and surgical therapy is at least partially effective in localized calcinosis.

Calcinosis in scleroderma.

To provide an update on the available literature regarding the epidemiology, pathophysiology, diagnosis, and treatment of calcinosis cutis in patients with systemic sclerosis (SSc). We identified observational studies that describe the frequency of calcinosis in SSc and associated clinical features; molecular studies exploring potential pathogenic mechanisms; and case reports and case series describing new diagnostic approaches and treatments. Calcinosis cutis is the deposition of insoluble calcium in the skin and subcutaneous tissues. It represents a major clinical problem in patients with SSc affecting at least one quarter of patients. It is associated with longer disease duration, digital ulcers, acro-osteolysis, positive anticentromere antibody, and positive anti-PM/Scl antibody. Although pathogenesis is unknown, there is evidence supporting local trauma, chronic inflammation, vascular hypoxia, and dysregulation of bone matrix proteins as potential mechanisms. Diagnosis can be made clinically or with plain radiography. Several pharmacologic therapies have been tried for calcinosis with variable and modest results, but surgical excision of calcium deposits remains the mainstay of treatment.

Topical sodium thiosulfate for the treatment of calcinosis cutis associated with autoimmune connective tissue diseases: The Mayo Clinic experience, 2012-2017

Topical sodium thiosulfate for the treatment of calcinosis cutis associated with autoimmune connective tissue diseases: The Mayo Clinic experience, 2012-2017

Calcinosis: pathophysiology and management.

This article reviews the most updated literature regarding the epidemiology, pathophysiology, diagnosis, and treatment of calcinosis cutis in patients with systemic sclerosis (SSc). Our review identified observational studies that describe the frequency of calcinosis in SSc and associated clinical features, genetic studies in animal models of heritable disorders leading to calcium deposition, and case series and case reports describing new diagnostic approaches and therapeutic interventions. Calcinosis cutis is the deposition of calcium in the skin and subcutaneous tissues. It affects almost one quarter of patients with SSc, and is associated with longer disease duration, digital ulcers, acroosteolysis, positive anticentromere antibody, and positive anti-PM/Scl antibody. Local trauma, chronic inflammation, and vascular hypoxia have been proposed as potential pathomechanisms. The development of mouse models that mimic heritable ectopic mineralization disorders are contributing to the understanding of the process of calcification. Diagnosis can be made clinically or with plain radiography. Experimental diagnostic studies include ultrasonography, multidetector computed tomography, and dual-energy computed tomography. Several pharmacologic therapies have been tried for calcinosis with variable results, but surgical excision of calcium deposits remains the mainstay of treatment.

Treatment of calcinosis cutis by extracorporeal shock-wave lithotripsy

Calcinosis cutis (CC) encompasses debilitating complications of connective tissue disorders and chronic venous insufficiency. Extracorporeal shock-wave lithotripsy (ESWL) is an effective treatment for urolithiasis, pancreatolithiasis, and calcified tendinitis. This study prospectively evaluated ESWL efficacy and tolerance for patients with CC. This monocentric prospective study included all consecutive patients with CC progressing for at least 3 months, while their underlying causal disease was not. They underwent 3 ESWL sessions at 3-week intervals. The CC area and associated pain (visual analog scale score and analgesic consumption) were recorded before and 6 months after ESWL. Eight patients were included: 4 with chronic venous insufficiency, 3 with systemic scleroderma, and one with dermatomyositis. ESWL was used to treat 10 CC lesions. Seven patients completed 3 ESWL sessions. Six months after ESWL, the median CC area had decreased from 3.1 to 1.9 cm(2). visual analog scale-assessed pain scores declined dramatically, from 7 to 2 of 10, as did analgesia consumption, without any difference according to the causal disease. Only 8 consecutive patients have been included and treated by ESWL during our study. This evaluation of ESWL efficacy and tolerance for the treatment of CC found no difference between the different underlying CC causal diseases in terms of efficacy. Based on our observations, ESWL efficacy was better against small, ulcerated, and radiopaque CC, and it had an analgesic effect that might make subsequent surgical excision of CC fragments easier. Ergonomic adaptations are required to facilitate and expand ESWL use in dermatology.

Surgical treatment of calcinosis cutis in juvenile dermatomyositis

Surgical treatment of calcinosis cutis in juvenile dermatomyositis

Extensive Calcinosis Cutis in Relapsed Acute Lymphoblastic Leukaemia

Introduction: Hypercalcaemia with calcinosis cutis occurring at relapse of acute lymphoblastic leukaemia (ALL) is rare and unusual. Clinical Picture: A 19-year-old lady with B precursor ALL presented with extensive waxy, verrucous, tender plaques over the flexures of her arms and legs a week after relapse of leukaemia. She was found to have hypercalcaemia, hyperphosphataemia, hyperuricaemia and acute renal impairment. Skin biopsy was consistent with calcinosis cutis. There was no evidence of metastatic calcification in other organs. Treatment: Hypercalcaemia was treated with aggressive hydration and intravenous pamidronate. High doses of analgesics were required for partial pain relief. Outcome: Cutaneous lesions proved resistant to early calcium lowering and were a source of constant pain. She succumbed to leukaemia four months later. Conclusion: Treatment of calcinosis cutis was unsatisfactory and would have been dependent on the successful treatment of the underlying leukaemia.