Calcinosis Research Articles

Idiopathic Localised Perforating Calcinosis Cutis Over the Bilateral Distal Phalanx of the Middle Finger - A Rare Case Report

Calcinosis cutis or cutaneous calcification occurs when calcium is deposited into the skin and subcutaneous tissue. It is classified into five primary types: Dystrophic, metastatic, idiopathic, iatrogenic and calciphylaxis. Idiopathic calcinosis cutis is a rare phenomenon that occurs in the absence of a known tissue injury or systemic metabolic defect. The various types of idiopathic calcinosis are idiopathic calcinosis of scrotum/penis/vulva, milia-like idiopathic calcinosis cutis, subepidermal calcified nodule, tumoural calcinosis, localised calcinosis cutis and calcinosis universalis. It is essential to delineate it from other calcification disorders for further plan of management. We report one such case of idiopathic localised perforating calcinosis cutis, symmetrically over a bilateral distal phalanx of the middle finger in a 72-year-old female.

Calcinosis and the cranium: inside and out.

Calcinosis and the cranium: inside and out.

Dermatomyositis with calcinosis universalis.

Dermatomyositis with calcinosis universalis.

Clinical and immunological characteristics of myositis complicated with thromboembolism

OBJECTIVE To investigate and analyse the clinical and immunological features of patients with myositis complicated with thromboembolism. METHODS We identified a cohort of 390 myositis patients diagnosed with myositis admitted to People's Hospital of Peking University from 2003 to 2019. The patients were retrospectively enrolled in this investigation. According to the outcome of the color Doppler ultrasound, CT pulmonary angiography, pulmonary ventilation and perfusion scan patients were divided into myositis with and without thromboembolism group. Demographic, clinical (heliotrope rash, Gottron's sign/papules, periungual erythema, skin ulceration, subcutaneous calcinosis, Mechanic's hands, myalgia, interstitial lung disease, pulmonary arterial hypertension), laboratory, immunological [anti-autoantibodies including melanoma differentiation associated gene 5 (anti-MDA5), anti-Mi-2, anti-transcription intermediary factor-1γ (anti-TIF-1γ, anti-nuclear matrix protein 2 (anti-NXP2), anti-small ubiquitin-like modifier activating enzyme (anti-SAE), anti-synthetase], imaging and therapeutic status data of the patients at the diagnosis of myositis with and without thromboembolism were collected and the differences in these data were analyzed. Logistic regressive analysis was used to identify the risk factors of thromboembolism. RESULTS In the retrospective study, 390 myositis patients were investigated. The mean age of onset was (49.6±13.4) years, male to female ratio was 0.31 :1. Thromboembolism was identified in 4.62% (18/390) of the myositis patients, which was lower than the published reports. Out of 18 patients with thromboembolism, 55.6% (10/18) of them were deep venous thrombosis, followed by cerebral infarction (22.2%, 4/18), pulmonary embolism (11.1%, 2/18), renal artery embolism (5.6%, 1/18) and embolism of upper extremity (5.6%, 1/18). Fifty percent of thromboembolism events occurred 6 months after the diagnosis of myositis, 38.9% of thromboembolism events occurred 6 months within the diagnosis of myositis, 11.1% of thromboembolism events occurred 6 months before the diagnosis of myositis. As compared with the myositis patients without thromboembolism, the myositis patients complicated with thromboembolism were older [(58.3±11.7) years vs. (49.3±13.4) years, P=0.006]. C-reaction protein (CRP) (12.2 mg/L vs. 4.1 mg/L, P < 0.001), ferritin (20 085.5 μg/L vs. 216.6 μg/L, P < 0.001) and D-dimer (529.0 μg/L vs. 268.0 μg/L, P=0.002) were significantly higher in thromboembolism group. Diabetes (44.4% vs. 16.4%, P=0.006), coronary heart disease (22.2% vs. 3.0%, P=0.003) and surgery (16.7% vs. 3.5%, P=0.032) were observed more common in thromboembolism group than those without thromboembolism. Activated partial thromboplastin time (APTT) (26.9 s vs. 28.7 s, P=0.049) and albumin (32.4 g/L vs. 36.5 g/L, P=0.002) was lower in thromboembolism group. The risk factors of thromboembolism in the myositis patients were low level of albumin (OR=0.831, 95%CI: 0.736-0.939, P=0.003), diabetes (OR=4.468, 95%CI: 1.382-14.448, P=0.012), and coronary heart disease (OR=22.079, 95%CI: 3.589-135.837, P=0.001) were independent significant risk factors for thromboembolism in the patients with myositis. There was no significant difference in clinical manifestations, myositis-specific antibodies or myositis-associated antibodies between the two groups. CONCLUSION Thromboembolism is a complication of myositis. Lower levels of albumin, diabetes, and coronary heart disease might be risk factors of thromboembolism in myositis patients.

Aggressive universal calcinosis complicating dermatomyositis

Aggressive universal calcinosis complicating dermatomyositis

Subcutaneous calcinosis: a rare complication of chronic hypercalcemia.

Subcutaneous calcinosis is a rare entity that may be secondary to either tissue damage, phosphocalcic disturbance or idiopathic origin. In the context of hypercalcemia, subcutaneous calcinosis remains a rare complication with few cases reported in the litterature. We report the case of a 27-year-old patient admitted for chronic hypercalcemia with right femur fracture and renal failure. Clinical examination found hard, painless, non-inflammatory subcutaneous nodules at the 2 elbows and at the lower gum (Panel A), the elbow radiography showed radiopaque structures in favor of subcutaneous calcinosis (Panel B). The etiological investigations of hypercalcemia showed a parathyroid hormon at 2200ng/ml (15-65), with cervical ultrasound showing a right inferior parathyroid nodule of 3cm * 1.6cm, in favor of primary hyperparathyroidism. The evolution was marked by the decrease of the size of the lesions in 15 days after normalization of the phosphocalcic balance after a symptomatic treatment based on intravenous rehydration, diuretics, corticosteroid bolus and bisphosphonate. Although hypercalcemia can reach very high serum levels in primary hyperparathyroidism, it is rarely accompanied by subcutaneous calcinosis. Indeed, it is hyperphosphoremia which represents the main factor involved in the development of cutaneous calcinosis in the context of phosphocalcic disturbances, and this independently of the level of hypercalcemia. Lesions tend to resolve spontaneously when calcium and phosphate levels normalize.

Effectiveness and safety of drug-eluting stent implantation following rotational atherectomy for severe coronary arteries calcification in elderly patients

Objective To investigate the effectiveness and safety of drug-eluting stent implantation following rotational atherectomy(RA)for severe coronary arteries calcification in elderly patients. Methods A total of 21 patients receiving RA and drug-eluting stent implantation were enrolled in this study in our cardiology department from Sep.2014 to Sep.2017.Twenty-one patients with 27 severe calcified lesions were treated with the stent implantation following RA.The primary endpoints of the study were the immediate operation success rate and the rate of major adverse cardiac and cerebral events(MACCE)at 6 month after surgery, including angina recurrence, need for target vessel revascularization, myocardial infarction, stent thrombosis and cardiovascular death. Results 14 patients(66.7%, 14/21)received RA by using 1.5 mm burr, and 7 patients(33.3%, 7/21)by using both 1.25 mm and 1.5 mm burrs.The average ratio of burr to artery diameter was(0.5±0.1). A total of 29 stents were successfully implanted in all patients(100%, 21/21 patients). None of the patients experienced any acute coronary artery rupture or other severe complications during percutaneous coronary intervention(PCI)after RA.Two cases(2/21, 9.5%)suffered from slow flow, and the coronary blood flow was restored to TIMI grade Ⅲ after treatment.The coronary blood flow in the other 19 cases(19/21, 90.4%)was TIMI grade Ⅲ after RA.Intravascular ultrasonography(IVUS)showed that the stents were well adhered without stent rupture and intimal tear in 12 cases(12/21, 57.1%), and postdilation was performed in 9 cases(9/21, 42.9%). All patients were followed up for at least 6 months, and target vessel revascularization and death were not found. Conclusions A drug-eluting stent implantation following rotational atherectomy is effective and safe for treating severe coronary arteries calcification in elderly coronary heart disease patients.The IVUS-guided rotational atherectomy combined with drug-eluting stent implantation can reduce the risk of MACCE, such as under-expansion stent, stent thrombosis, myocardial infarction, cardiovascular death, and improve clinical outcomes in elderly patients with severe coronary arteries calcification. Key words: Coronary artery disease; Calcinosis; Drug-eluting stent

Preliminary application of susceptibility weighted imaging in meningiomas

Objective To evaluate the preliminary application of susceptibility weighted imaging (SWI) in meningiomas. Methods Fifty meningioma patients, treated at Department of Neurosurgery of Nanjing Brain Hospital Affiliated to Nanjing Medical University between October 2015 and February 2018, were retrospectively enrolled into this study. All cases underwent brain CT, MRI and SWI scans preoperatively, and the minimum intensity projection (mIP) was obtained through processing basic SWI images by software. Compared with MRI and CT, intratumoral microhemorrhage, calcification and drainage vein in meningioma were analyzed in mIP and filtered phase imaging. Pathological examination was carried out after operation. Further comparative analysis was accomplished between microhemorrhagic group and non-microhemorrhagic group in imaging features and pathological indexes. Results Among the 50 meningioma patients examined by SWI, 7 (14%) had 24 intratumoral microhemorrhages; 24 (48%) had 47 intratumoral calcifications, including 31 calcifications that were consistent with CT and 16 which were not detected by CT; 16 (32%) clearly showed dominant draining veins originating from the tumor itself, consisting of 11 without peritumoral edema and 5 with peritumoral edema. The patients without peritumoral edema were more likely to form drainage veins than those with peritumoral edema [61%(11/18) vs. 16% (5/32), P=0.001]. All 50 patients were pathologically confirmed to have WHO Grade Ⅰ meningiomas. The edema index (EI), maximum edema width and Ki67 in the microhemorrhagic group were significant higher than those in non-microhemorrhagic group (all P 0.05). Conclusions Intratumoral microhemorrhage, microcalcification and drainage vein can be detected by SWI. Intratumoral hemorrhages infrequently occur in WHO Grade Ⅰ meningiomas. Microhemorrhagic meningiomas have higher Ki67 and more serious peritumoral edema than non-hemorrhagic ones. The efferent draining vein from meningioma itself may alleviate the formation of peritumoral edema. Key words: Meningioma; Calcinosis; Susceptibility weighted imaging; Microhemorrhage; Vein

Calcinosis of the Mandible in Dermatomyositis

Calcinosis of the Mandible in Dermatomyositis

Clinical characteristics of biopsy-proven renal sarcoidosis in Japan.

Background: Although some articles have described renal sarcoidosis, the incidence among biopsy cases remains unclear. Here, we defined the incidence of renal sarcoidosis among renal biopsy cases and analyzed the clinical course. Methods: We performed an epidemiological study examining renal biopsy cases treated at 5 centers between January 2000 and September 2015 and identified 16 cases (7 men, 9 women; mean (±SD) age, 59.4±18.6 years) out of a total of 14191 renal biopsy cases. Renal involvement of sarcoidosis was defined as granulomatous tubulointerstitial nephritis, tubulointerstitial nephritis without granulomatous lesions, and renal calcinosis. Fifteen of the cases were treated with steroid therapy. One case initially received steroid pulse therapy. The outcome was evaluated based on the estimated glomerular filtration rate (eGFR), CKD stage, and the change in eGFR (ΔeGFR) after treatment. A favorable response was defined as ΔeGFR ≥25%. Results: The incidence of renal sarcoidosis was 0.11%. The mean eGFR was 28.2±16.1 mL/min/1.73 m2. At the last observation, the mean eGFR was 43.7±19.7 mL/min/1.73 m2. Although a favorable response to steroid therapy was found in the majority of cases (10/15, 67%), 12 of the 15 cases (80%) had residual renal dysfunction at the last observation and 8 cases (53%) had moderate to severe renal dysfunction. Conclusion: Renal sarcoidosis is extremely rare among renal biopsy cases. Among cases with an unfavorable response to steroid therapy, pathogenetic mechanisms other than sarcoidosis and severe nephron damage were observed. (Sarcoidosis Vasc Diffuse Lung Dis 2018; 35: 252-260).

037 Dermatomyositis and calcinosis

037 Dermatomyositis and calcinosis

The relationship of IL-6, hs-CRP and Fetuin-A with cardiac valve calcification in maintenance hemodialysis

Objective To investigate the relationship of IL-6, hs-CRP and Fetuin-A with cardiac valve calcification(CVC) in maintenance hemodialysis(MHD). Methods A total of 163 ca-ses of MHD patients in our hospital from January 2014 to December 2016 were collected, according to the whether ornot CVC were divided into the CVC group(n=68) and the non-CVC group(n=95), and according to the number and extent of CVC were divided into the non-CVC group(n=95), one CVC group(n=37), two CVC group(n=31) and mild group (n=22), moderate group (n=31), severe group (n=15). The levels of IL-6, hs-CRP and Fetuin-A in each group were compared, the correlation between Fetuin-A and clinical data was analyzed. Results In the 163 patients, CVC was found in 68 patients(41.72%). The abdominal aorta calcification score, dialysis time, age, iPTH, hs-CRP, IL-6, serum phosphorus, calcium phosphorus product in the CVC group were higher than non-CVC group, the serum albumin, Fetuin-A were lower than non- CVC group(P 0, P<0.05), and negative correlation with abdominal aorta calcification score, CVC, IL-6, hs-CRP, iPTH, dialysis time and age reached significant level(r<0, P<0.05). Conclusions The severity of CVC in patients with CVC is correlated with abdominal aorta calcification score, IL-6, hs-CRP and Fetuin-A, and has some clinical value in evaluating the occurrence and development of CVC in patients with MHD. Key words: Renal Dialysis; Heart Valves; Calcinosis; Interleukin-6; C-Reactive Protein; alpha-Fetoproteins

Imaging Features of Soft-Tissue Calcifications and Related Diseases: A Systematic Approach

Soft-tissue calcification refers to a broad category of lesions. Calcifications are frequently identified by radiologists in daily practice. Using a simple algorithm based on the distribution pattern of the lesions and detailed clinical information, these calcified lesions can be systematically evaluated. The distribution pattern of the calcific deposits enables initial division into calcinosis circumscripta and calcinosis universalis. Using laboratory test results (serum calcium and phosphate levels) and clinical history, calcinosis circumscripta can be further categorized into four subtypes: dystrophic, iatrogenic, metastatic, and idiopathic calcification. This pictorial essay presents a systematic approach to the imaging features of soft-tissue calcifications and related diseases.

Relationships of ductal carcinoma in situ calcification feature and ER, PR and HER2 expression

Objective To discuss the relationships of ductal carcinoma in situ calcification feature and estrogen receptor (ER), progesterone receptor (PR), human epidermal growth factor receptor 2 (HER2) expression. Methods The mammary gland molybdenum target X-ray results of 226 patients with ductal carcinoma in situ were retrospectively analyzed in our hospital during January 2013 to December 2016. All patients were divided into calcification group (n=110) and no calcium group (n=116). Immunohistochemical method was used to detect the expressions of ER, PR and HER2, and their relationships with calcification feature were analyzed. Results The positive expression rates of ER and PR in calcification group were 72.73% and 54.55%, significantly higher than those in no calcium group (39.66% and 38.79%; χ2=25.033, P 30, with mass were the independent prediction factors of ER, PR and HER2 expressions (all P<0.001). Conclusion The mammary gland molybdenum target calcification features of ductal carcinoma in situ are associated with ER, PR and HER2 expressions, the small polymorphic form, cluster distribution, number>30, with mass can be used as important reference indexes to preliminarily predict biological therapy and expressions of prognostic factors. Key words: Carcinoma, intraductal, noninfiltrating; Calcinosis; Receptors, estrogen; Receptors, progesterone; Receptor, epidermal growth factor

A case of tumoral calcinosis

A case of tumoral calcinosis

Treatment of Calcinosis Cutis with Minocycline in Five Dogs

Treatment of Calcinosis Cutis with Minocycline in Five Dogs

Profile of Pediatric Idiopathic Inflammatory Myopathies from a Tertiary Care Center of Eastern India.

To report data on Idiopathic inflammatory myopathies (IIM) from eastern India. All IIM patients diagnosed over the last 5 y (2011-2016) were included through a retrospective review of records from the hospital and specialty clinic at Institute of Postgraduate Medical Education & Research (I.P.G.M.E.&R.), Kolkata. Out of the 11 IIM patients, 9 had Juvenile dermatomyositis (JDM) and 2 had overlap myositis (OM) [with systemic lupus erythematosus (SLE) and scleroderma]. The overall sex ratio was M: F=1: 2.6. The mean age at diagnosis was 6.94 y for JDM and 7 y for OM. The mean interval from onset to diagnosis was 5.2 mo. All patients had heliotrope rash and proximal myopathy (n=11,100%). Other findings included Gottron papule (n=7; 64%), arthritis (n=6; 54%), malar rash (n=5; 45%), dysphagia (n=4; 36%), nasal intonation (n=3; 27%), subcutaneous nodule (n=2; 18%), cutaneous sinus (n=1; 9%), calcinosis universalis (n=1; 9%), GI bleed (n=1; 9%). All patients had raised erythrocyte sedimentation rate (ESR), aspartate aminotransferase (AST), lactate dehydrogenase (LDH) while 10 had raised creatine kinase (CK). Three were anti-nuclear-antibody (ANA) positive. Electromyography (EMG) showed proximal myopathy in most cases (n=9; 82%). All patients received corticosteroid. Intravenous immunoglobulin (IVIG) was given to 2 patients. Two received hydroxychloroquine. Cyclophosphamide and azathioprine were given in one each. This study, first reported profile of IIM from eastern India, showed JDM as the commonest form of IIM with a female preponderance. Five children had complete and 2 had partial remission. Two patients of JDM and 1 of OM died. Increased awareness, early referral, prompt diagnosis and treatment might improve the outcome and survival.

Dermatological manifestations in chronic renal failure patients with and without hemodialysis: A study at a tertiary care centre

Background: Skin is an important diagnostic window to many diseases of internal organs including the renal system. Subtle changes in the skin may act as a clue to the underlying renal pathology. The cutaneous manifestations may be caused either by the disease or drugs used in the treatment of chronic kidney disease (CKD). Aim: The aim of this study is to investigate and compare cutaneous manifestations in patients with CKD on hemodialysiswith patients not on dialysis. Materials and Methods: One hundred patients with CKD were recruited, 50 each in maintenance hemodialysis (MHD) and nondialysis group. Detailed cutaneous examination and necessary investigations were done, and cutaneous manifestations were compared among the two groups. Results: Of the 100 patients, 72 were males and 28 were females. Mean duration of disease was 32.4 months in the dialytic group and 11.2 in the nondialytic group. The most common underlying disease that lead to CKD was diabetes mellitus. Specific cutaneous manifestations observed were Kyrles disease 4%, calcinosis cutis 1%, and uremic frost in 1%. The common nonspecific manifestations observed were xerosis in 59% (dialytic 61.03%, nondialytic 38.98%), pallor in 57% (dialytic 49.1%, nondialytic 50.9%), pruritus in 38%, (dialytic 63.16%, nondialytic 36.84%), pigmentation in 32% (dialytic, 65.63%, nondialytic 34.37%), purpura in 8% of dialytics, and none of the nondialytic patients had purpura. Cutaneous infections were found in 28%, of which fungal were 20%, bacterial 7%, and viral infections 2%. Nail changes were observed in 46% (dialytic 62%, nondialytic 30%), hair changes in 16%, and mucosal manifestations in 11% of the patients. Puncture marks were found in 74% of the dialytic patients. Xerosis, pruritus, and pigmentation had significantly higher prevalence in diabetic uremics compared to nondiabetic uremics. Conclusions: There was a significant association with the mean duration of disease, which is higher in dialytic patients compared to the nondialytic group. Xerosis, pruritus, and hyperpigmentation were common in dialytic patients.

Nephrocalcinosis in childhood

Nephrocalcinosis (NC) describes the deposition of calcium-oxalate or calcium-phosphate crystals in the tubuli or interstitial kidney parenchyma. Whereas kidney stones are formed in every age NC is most frequently seen during the first years of life. Because of multiple causative factors NC is a diagnostic challenge. A mostly metabolic reason for the development of nephrocalcinosis can be identified in up to 75% of patients and hence adequately be treated. An imbalance of lithogenic and inhibitory substances is the pathophysiologic basis for the development of nephrocalcinosis. This imbalance is either transient, for example in patients with alimentary hyperoxaluria or short term immobilization induced hypercalciuria, or ongoing, e.g., in tubulopathies or inborn errors of metabolism. Hypercalciuria is the most prominent promotor for the development of nephro- calcinosis, either accompanied by hypercalcemia or not. Both hyperoxaluria, in its primary or secondary forms, as well as hypocitraturia, e.g., in renal tubular acidosis, or in the premature infant, are further specific risk factors. Of course, although mostly clinically silent, early diagnosis is mandatory to start treatment, which may help not only to prevent progression of NC but end stage renal failure relying on the underlying diseases.

Comparing Three Drug Regimens for Juvenile Dermatomyositis

Juvenile onset of dermatomyositis is associated with risk for permanent muscle loss, weakness, and the possibility of calcinosis if not treated early.