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PubMed2003

Idiopathic circumscripta calcinosis cutis of the knee.

BACKGROUND: Calcinosis cutis, a disease characterized by the presence of calcium deposits in the skin, is classified into four types according to etiology: dystrophic, metastatic, iatrogenic and idiopathic. The dystrophic form is the most common while the idiopathic one is the rarest, but specific incidence and frequency data are not available in scientific literature. OBJECTIVE: Calcinosis cutis circumscripta is a very rare form of idiopathic calcinosis cutis arising in the second half of life. It typically involves the extremities and is associated with prior trauma and scleroderma. We dealt with a very rare form of calcinosis cutis circumscripta in a healthy patient, for whom surgical excision revealed to be an effective and successful treatment. METHODS AND MATERIALS: We present the case of a 46-year-old woman affected by idiopathic circumscripta calcinosis cutis of the left knee, successfully treated by surgical removal. DISCUSSION: Medical and surgical treatment are options to cure calcinosis cutis. Medical therapy is not very effective. Surgical excision has shown to be beneficial, as it can provide a symptomatic relief. However, since calcinosis cutis limits are not always well defined a recurrence of the lesions may occur.

Adult↗
PubMed2003

Treatment of cutaneous calcinosis in limited systemic sclerosis with minocycline.

OBJECTIVES: To evaluate the effect of minocycline as treatment for cutaneous calcinosis in limited cutaneous systemic sclerosis (lcSSc). METHODS: Patients with lcSSc who had cutaneous calcinosis causing pain or ulceration, or both, were prescribed minocycline 50 or 100 mg daily regularly in an open label manner between November 1994 and April 2000. At routine clinical follow up the appearance of the calcinosis deposits was assessed clinically and radiographically, and the patients' assessment of the degree of discomfort, size, and frequency of ulceration was recorded. Demographic data, including disease duration, clinical features, and antinuclear antibody (ANA) titres, were also recorded. RESULTS: Nine patients have been treated to date. Eight of the nine patients were ANA positive, five of whom were positive for anticentromere antibodies. Eight patients have shown definite improvement and seven patients continue to receive treatment. The frequency of ulceration and inflammation associated with the calcinosis deposits decreased with treatment. The size of the calcinosis deposits also decreased but was less dramatic than expected. Improvement occurred at the earliest after one month of treatment with a mean (SD) of 4.8 (3.8) months. The mean (SD) length of treatment was 3.5 (1.9) years. An unexpected effect was the darkening of the calcinosis deposits to a blue/black colour. CONCLUSIONS: Minocycline may be effective in the control of calcinosis in systemic sclerosis. A low dose only is required and appears to be generally well tolerated. The mechanism of action may be mainly through inhibition of matrix metalloproteinases and anti-inflammatory effects. Calcium binding properties and antibacterial actions may also have a role.

Adult↗
PubMed1992

[Patients with calcinosis cutis in National Leprosarium Matsuoka Hoyo-En].

As of the year 1991 there were 358 leprosy patients in National Leprosarium Matsuoka Hoyo-En, including 223 patients (62.3%) who had received the injections of chaulmoogra oil before. Calcinosis cutis caused probably from the injections was noted on 73 patients (32.7%): 67 lepromatous and 6 tuberculosis cases. It has never been reported before that the T type patient suffering from calcinosis cutis was observed in the cases of the chaulmoogra oil injection in Japan. The detectable positions of calcinosis cutis were mostly at the injected sites, that is, outside the right brachium followed by bilateral-branchia and crura. In the group of patients with calcinosis cutis, the anti-PGL antibody was negative for the most part. Urinalyses, peripheral blood figure analyses, histopathological tissue examinations of calcium deposition, X-ray diffraction patterns, differential thermal and gravitational analyses, and chemical analyses were performed on all patients with this disease. Further, as the result of this study six patients with calcinosis cutis caused by sulpyrine was also found. The major component of the deposit by the drug was calcium phosphate. These calcinosis cutis were considered to be of trophopathic calcinosis based on the disorder of subcutaneous tissue due to the injections of respective drugs: chaulmoogra oil and sulpyrine.

Calcinosis↗
PubMed1975

[Calcinosis of the cardiac coronary arteries].

In patients who had died due to various diseases and also in practically healthy individuals aged from 10 to 79 years calcinosis of the coronary arteries was studied morphometrically and histologically. The incidence and the extend of calcinosis implicating the coronary arteries increased with growing age and were more pronounced in males than in females. In analysis of observations uncovered, irrespective of the cause responsible for death, a direct relationship between the area of calcinosis and that of atherosclerosis in grosso modo (correlation factor of 0.46) was noted; the diseased in consequence of diverse affections demonstrated considerable variations in the development of arterial calcinosis. More often than not calcinosis of the coronary arteries was definable in atherosclerotic plaques carrying an important fibrotic component, in cases of necrosis and in those marked by a low lipids content and poor vasculalarization of an altered wall of the coronary artery. Calcinosis of the coronary arteries occurred more often than did their stenosis on 50% or more of the lumen. There has been established a direct relation between the frequency of the coronary artery stenosis and the area of calcinosis. The latter usually appears as a sign of disseminated atherosclerosis rather than as that of a progressive atherosclerotic process.

Adult↗
PubMed2000

Tumoral calcinosis of the lumbar meninges: case report.

OBJECTIVE AND IMPORTANCE: Tumoral calcinosis is a rare disorder of unknown origin. Tumoral calcinosis involving the spine is extremely rare. This is the first case of tumoral calcinosis localized in the dura mater of the lumbar spine. CLINICAL PRESENTATION: This 55-year-old male patient presented with tumoral calcinosis of the lumbar meninges. T1-weighted, sagittal magnetic resonance imaging scans of the lumbar spine revealed a round mass of slightly increased intensity with high-intensity margins, located posterior to the cauda equina at the L4-L5 level. The cauda equina was severely compressed anteriorly. T2-weighted scans revealed that the mass was composed of a high-intensity area with low-intensity margins. T1-weighted magnetic resonance imaging scans obtained after intravenous gadolinium administration revealed some enhancement at the margins. INTERVENTION: The mass lesion was totally resected. Three years after surgery, no recurrence was observed in follow-up magnetic resonance imaging scans. CONCLUSION: Although tumoral calcinosis is a rare cause of mass lesions of the lumbar spine, it should be considered in differential diagnoses. If the lesion can be totally resected, the prognosis should be good, similar to that for general tumoral calcinosis.

Calcinosis↗
PubMedDate not supplied

Detection of cardiac calcinosis in hemodialysis patients by whole-body scintigraphy with 99m-technetium methylene diphosphonate.

A noninvasive method for the diagnosis of cardiac calcinosis, a life-threatening complication in hemodialysis patients with end-stage renal disease (ESRD), has not, as yet, been firmly established. We tested whether whole body scanning with 99m-technetium methylene diphosphonate (MDP) might visualize cardiac calcinosis. In 19 consecutive chronic hemodialysis ESRD patients (13 males and 6 females, aged 40-81, mean 63 +/- 8 years) with cardiovascular disease [mitral annular calcinosis and/or calcified aortic valve (n = 4), hemodialysis cardiomyopathy (n = 1), coronary artery disease (n = 9) and peripheral artery atherosclerotic disease (n = 6)], MDP uptake in the heart was compared to that in 7 non-ESRD controls with hyperparathyroidism due to adenoma. Cardiac and lung field MDP uptake was confirmed in only 3 (16%) and 5 (26%) of the 19 ESRD subjects, respectively, but was absent in controls. Positive cardiac uptake was related to cardiac calcified complications (mobile intracardiac calcinosis, myocardial calcinosis and mitral annular calcification) and the duration of hemodialysis (p = 0.015). While it was statistically insignificant, subjects showing MDP uptake were elder and had higher serum Ca or Ca x P product and lower intact parathyroid hormone levels. These results suggest that cardiac calcinosis in ESRD patients can be detected noninvasively by myocardial scintigraphy with 99m-technetium MDP.

Adult↗
PubMed2005

A novel mutation in fibroblast growth factor 23 gene as a cause of tumoral calcinosis.

CONTEXT: Tumoral calcinosis is a disease characterized by ectopic calcification and hyperphosphatemia due to enhanced renal tubular phosphate reabsorption. Fibroblast growth factor (FGF)23 was identified as a responsible factor in hypophosphatemic diseases caused by renal phosphate leak. OBJECTIVE: The objective of the study was to analyze the involvement of FGF23 in the development of tumoral calcinosis. DESIGN: Serum FGF23 level was evaluated in a patient with tumoral calcinosis by two kinds of ELISA: full-length assay that detects only full-length FGF23 with phosphate-lowering activity and C-terminal assay that measures full-length as well as C-terminal fragment of FGF23. FGF23 gene was analyzed by direct sequencing of PCR products, and mutant FGF23 was analyzed by Western blotting after expression in mammalian cells. PATIENTS: A family of tumoral calcinosis patients were studied. RESULTS: Serum FGF23 was extremely high when measured by C-terminal assay. In contrast, it was low normal by full-length assay. Analysis of FGF23 gene detected a serine to phenylalanine mutation in codon 129. No wild-type allele of this codon was found in the patient. The brother of the proband showed the same base change. When this mutant FGF23 was expressed in vitro, full-length and N-terminal fragments were barely detectable by Western blotting, whereas C-terminal fragment with the same molecular weight as that from wild-type FGF23 could be detected. CONCLUSION: The production and serum level of C-terminal fragment of FGF23 are increased in this patient with tumoral calcinosis. Together with the recent similar report of FGF23 mutation, impaired action of full-length FGF23 seems to result in tumoral calcinosis.

Adult↗
PubMed2006

Efficacy of probenecid for a patient with juvenile dermatomyositis complicated with calcinosis.

Calcinosis of juvenile dermatomyositis (JDM) is a crucial problem because it is refractory to various therapies. An 11-year-old boy who had been treated for JDM with interstitial pneumonia developed calcinosis of both legs despite treatment with corticosteroid and cyclosporin A. Images of his knees showed massive calcinosis with restricted range of motion. Probenecid was used to reduce calcinosis, resulting in remarkable improvement of calcinosis accompanied by normalization of serum phosphorus level and disability after 17 months of administration. We suggest that probenecid is useful for the treatment of calcinosis of JDM.

Anti-Inflammatory Agents↗
PubMed1979

Cutaneous manifestations of tumoral calcinosis.

We have followed up a large family in which seven members have tumoral calcinosis. One girl had the skin lesions of localized calcinosis cutis apart from the typical subcutaneous deposits of calcium. Like most persons with tumoral calcinosis, our patient had normal serum calcium concentrations; however, the serum phosphorus levels were greatly elevated. The familial occurrence and elevated serum phosphorus levels suggest the possibility of some as yet undefined, heritable metabolic defect as the underlying cause. The occurrence of tumoral calcinosis with localized calcinosis cutis is a rare association, and there has been only one other reported case to our knowledge. This report describes our patient and offers a brief discussion of tumoral calcinosis. The therapeutic response to the phosphate depletion regimen and topical steroids was disappointing in our case.

Calcinosis↗
PubMed2005

Calcinosis cutis universalis in a patient with systemic lupus erythematosus.

Deposition of calcium salts in the skin and subcutaneous tissue occurs in a variety of rheumatic diseases, being most commonly associated with scleroderma, CREST (calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia), dermatomyositis, and overlap syndromes but is a rare complication of systemic lupus erythematosus (SLE). Calcinosis is classified into four subsets: dystrophic, metastatic, idiopathic, or calciphylaxis/iatrogenic. The pathophysiology of calcinosis cutis remains unclear. Our patient developed extensive areas of calcifications in the trunk and extremities (calcinosis universalis) 8 years after SLE diagnosis, which would correspond to a form of dystrophic calcification. No response was observed after treatment with oral diltiazem for 3 months. We review the literature on the pathogenesis and prevalence of calcinosis universalis in SLE.

Adult↗
PubMed1985

Oral lesion in a patient with calcinosis and arthritis: case report and differential diagnosis.

Calcinosis, the process whereby calcium salts are deposited in soft tissues, may be idiopathic, metastatic or dystrophic. Metastatic calcinosis develops in a variety of systemic diseases characterized by either hypercalcemia, hyperphosphatemia, or both. Dystrophic calcinosis refers to calcification of previously damaged or necrotic tissue. It may be found accompanying inflammatory or degenerative conditions and is frequently associated with connective tissue diseases. When pathologic calcification is widespread, an attempt must be made to determine the underlying cause. A case is presented in which there was multifocal calcium deposition in soft tissues, including an intra-oral site. The patient also exhibited severe arthritis, sicca syndrome, focal alopecia and vitiligo. In view of this clinical spectrum, one of the "collagen diseases" (dermatomyositis, lupus erythematosus, rheumatoid arthritis, and scleroderma) was suspected as a predisposing factor for disseminated calcinosis. When diagnostic workup failed to reveal a specific connective tissue disease, it was concluded that "undifferentiated connective tissue disease" was responsible for dystrophic calcinosis.

Aged↗
PubMed2004

Low dose warfarin treatment for calcinosis in patients with systemic sclerosis.

OBJECTIVE: To evaluate the effect of low doses of warfarin in patients with systemic sclerosis with disseminated subcutaneous calcinosis. METHODS: Three patients with disseminated subcutaneous calcinosis were treated with low doses of warfarin for 1 year. Subcutaneous calcinotic lesions, coagulation blood parameters, and the tendency for bleeding were followed up during the year. RESULTS: Two of the patients, who had newly diagnosed, diffuse, and relatively small calcinotic lesions, responded to warfarin treatment, with complete resolution of the calcinosis. The other patient, with larger and longer standing calcinotic lesions, did not respond to warfarin treatment. None of the three patients showed a prolongation of prothrombin time or partial thromboplastin time, nor did any have an increased tendency for bleeding. CONCLUSIONS: Low dose warfarin may serve as an effective treatment for calcinosis in a selected group of patients who have small and relatively new onset calcinosis. This treatment does not prolong the coagulation of blood and there is no increased tendency for bleeding.

Adult↗
PubMed1997

Paraspinal calcinosis associated with progressive systemic sclerosis. Case report.

The authors describe a case of paraspinal calcinosis in a 65-year-old woman with progressive systemic sclerosis. Although calcinosis occurs in up to 27% of cases of progressive systemic sclerosis, symptomatic paraspinal calcinosis is extremely rare. In the case reported here, multiple cervical facet joints were compromised by progressive calcinosis, leading to glacial spinal instability. Internal fixation was indicated to correct the instability and decompress the spinal canal. Medical therapy was instituted to arrest or reverse the ongoing calcinosis.

Aged↗
PubMed2006

Tumoral calcinosis in bilateral facet joints of the lumbar spine in scleroderma. Case report.

Tumoral calcinosis commonly occurs in the articular soft tissues of the extremities but rarely in the spine. The authors performed surgery to treat lumbar tumoral calcinosis in a patient with scleroderma, in whom symptoms of neurological dysfunction had manifested. This 49-year-old woman presented with low-back pain and gait disturbance. Seven years before presentation, scleroderma had been diagnosed, and the patient had received medical treatment ever since. Imaging revealed tumoral calcinosis centered at the bilateral facet joints between L-3 and L-4, marked stenosis of the spinal canal, L-3 spondylolisthesis, and intervertebral instability. Surgery was performed to excise the lesion en bloc. After neural decompression, posterolateral fusion and pedicle screw fixation were undertaken. Symptoms improved after surgery. In this case, the underlying scleroderma that predisposes to calcinosis and facet joint degeneration due to lumbar spondylolisthesis were probably factors leading to the development of tumoral calcinosis in the lumbar spine.

Calcinosis↗
PubMed1993

[Cutaneous or subcutaneous calcinosis observed in leprosy patients. 1. Clinical observations].

A roentgenographic examination was made for the limbs of leprosy patients with calcinosis in whom atrophic cutaneous sclerosis and subcutaneous induration or infiltration were observed. The observation results are summarized as follows. 1. Atrophic cutaneous sclerosis was one of a sequela in lepromatous lesion, especially in case of ENL, and it was observed to occur frequently at the extended sides of 1/3distal part from the forearm and the crus mainly. The atrophied cutaneous surface was tinged with lustrous red. It was able to observe calcium deposition directly just under the skin and/or in the shallow subcutaneous region from the roentgenogram of the site. The roentgenographic patterns were demonstrated as if many granules were scattered, and also the dendric and reticular platy-expansions were detected in some cases. The enucleated parts seemed to be similar to the cancellous bone. It might be said that dystrophic calcinosis cutis developed by inducing histological disorder is one of the origin of such a calcinosis, because the skin in these regions is deficient in the mobility and tends to provoke the circulatory disorder in case of chronic inflammation as discerned in lepromatous lesion. 2. An induration in subcutaneous tissue is lipid lump being as it was when chaulmoogra oil was injected and not undergo absorption of the oil. The lipid lumps enveloped in the tunic were observed in the site of lateral upperarm and the front of femur. They seemed to be remained almost all as it was. It was observed that the lipid lumps, as such, were adjacent to the outer layer of fascia, but not in the muscle. And there are some cases where the oil flowed from the injection site through the hypodermis and got the lipid lumps formed in the forearm and/or the crus. Roentgenogram of that showed the existence of calcinosis regardless of size which transmissivity of X-ray had an irregular pattern. The enucleated lipid lumps were easily cut to pieces by scalpel. 3. It may be said that the calcinosis observed in atrophic cutaneous sclerosis due to lepromatous lesion or lipid lump of unabsorbed chaulmoogra oil makes it necessary for its healing to be 10-20 years. 4. Roentgenogram at that time revealed no abnormality as to serum calcium, phosphorous and/or alkaline phosphatase values.

Adult↗
PubMed1999

[Compression of the sciatic nerve in uremic tumor calcinosis].

Tumoral calcinosis is an uncommon and benign condition characterized by the presence of slow-growing calcified periarticular soft tissue masses of varying size. They are usually asymptomatic and nerve compression is rare. We describe the case of a 54-year-old female patient on long-term hemodialysis for chronic renal failure presenting sciatica in the left lower limb secondary to an extensive uremic tumoral calcinosis that affected the hip and thigh. The pathogenesis of uremic tumoral calcinosis as well as the treatment and clinical outcome are analyzed. The uncommon nerve compression due to tumoral calcinosis are reviewed. In conclusion, uremic tumoral calcinosis is a not previously reported infrequent cause of sciatic nerve compression.

Calcinosis↗
PubMed1985

[Calcinosis universalis with hyperphosphataemia--successful treatment with phosphorus deprivation].

Seven year old boy with calcinosis universalis associated with high serum phosphorus was reported. Over one year's treatment with aluminum hydroxide up to 18 grams per day have reduced calcified mass remarkably, while serum phosphorus levels were remaining still above the normal range. Concomitant use of probenecid seemed to be effective in reducing serum phosphorus levels as well as decreasing the calcified mass. Pathogenetic mechanisms of soft tissue calcification in calcinosis universalis is not clear, but in this case hyperphosphataemia is considered to be one important factor accelerating soft tissue calcification. Although hyperphosphataemia associated with tumoral calcinosis has been frequently observed, no report is yet available on calcinosis universalis associated with hyperphosphataemia. This case might represent one unique type of calcinosis.

Aluminum Hydroxide↗
PubMed1985

[Diagnosis of aortic valve calcinosis with a view to the surgical treatment of aortic valve disease].

Combined roentgenologic/echocardiographic diagnosis of aortic valve calcinosis was made in 135 patients operated on for a rheumatic aortic heart disease. Four degrees of aortic valve calcinosis were identified: 1) isolated fine points of calcinosis, as revealed by echolocation and specific treatment of the removed valves; 2) small-focal calcinosis as revealed roentgenologically in 79.2% of cases and by echolocation, in 93%; 3) large-focal, and 4) wide-spread calcinosis, the latter two being detectable both roentgenologically and echocardiographically in all cases. The 4-degree classification of aortic valvular lesions allows a detailed assessment of the nature of the affection that is important for the choice of surgical procedure.

Aortic Valve↗