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Calcinosis Universalis

CPXD


Presentation

  • Dermatomyositis sine myositis presenting with calcinosis universalis SIR, Dermatomyositis can initially present without muscle involvement.[deepdyve.com]
  • The authors present the case of a 25 year-old female diagnosed with dermatomyositis who developed calcinosis universalis after stopping therapy.[ncbi.nlm.nih.gov]
  • It is classified into four types according to etiology, namely as dystrophic if calcium and phosphorus levels are normal and tissue damage is present, as idiopathic if calcium and phosphorus levels are normal and no tissue damage is present, or as metastatic[karger.com]
  • The purpose of this paper is to present a case of a rare condition and a treatment which yielded satisfactory results. The case was observed at frequent intervals for a period of four years.[jamanetwork.com]
  • We report the case of a woman presenting calcinosis cutis universalis complicating a lupus/scleroderma overlap syndrome, treated with zoledronate. Case report: A 41-year-old woman, with a history of SLE diagnosed in 1990.[morressier.com]
Dentist
  • Accuracy of entries is verified by an expert review board including dentists and dental hygienists. Colored thumb bleeds make it easy to locate definitions quickly.[books.google.com]
Respiratory Distress
  • The patient denied any history of fever, joint pains, weakness or pain in the muscles around shoulder and hip, pain or bluish discolouration of extremities, chest pain or respiratory distress. There was no family history of a similar illness.[ijdvl.com]
Gagging
  • Akkari; Gilberto Waisberg; Clóris Kessler; Tabata de Alcantara; Patrícia L Delai Related Documents : 8206076 - Vulvar intraepithelial neoplasia of low grade: a challenging diagnosis. 8367356 - Acute urinary retention in children. 9533356 - Psychogenic gagging[biomedsearch.com]
Oral Ulcers
  • There was no facial erythema, oral ulceration, finger tip ulceration or scars, nail fold capillaries, sclerodactyly and telangiectasia. Raynaud's phenomena could not be elicited.[ijdvl.com]
Myopathy
  • Chester V Oddis, Idiopathic inflammatory myopathy: management and prognosis, Rheumatic Disease Clinics of North America, 28, 4, (979), (2002). Ann M.[doi.org]
  • Inflammatory myopathies: Part 1. Ann Neurol. 1985;17:215-27. [ pubmed/open access ] [ crossref ] 5. Mastaglia FL, Ojeda VJ. Inflammatory myopathies: Part 2. Ann Neurol. 1985;17:317-23. [ pubmed/open access ] [ crossref ] 6. Miller FW.[jped.com.br]
  • Introduction Juvenile dermatomyositis (JDM) is a rare disease but it is the most common inflammatory myopathy in children. It is an autoimmune vasculopathy which primarily affects skin and proximal striated muscles.[scirp.org]
  • This is usually combined with a steroid-sparing agent, as steroid-induced myopathy can occur after four to six weeks of therapy (Table 3).[woundsresearch.com]
Hip Pain
  • The patient denied any history of fever, joint pains, weakness or pain in the muscles around shoulder and hip, pain or bluish discolouration of extremities, chest pain or respiratory distress. There was no family history of a similar illness.[ijdvl.com]
Malar Rash
  • She had a malar rash and widespread hyperpigmented skin lesions with palpable hard deposits in the soft tissue.[sajr.org.za]
Confusion
  • Clinically, it may be confused with a diagnosis of lupus panniculitis, and a biopsy is essential to confirm the diagnosis. It may be a source of pain and significant disability; no effective therapy is available at present. 1.[sajr.org.za]

Workup

Soft Tissue Calcification
  • No cause could be found for the soft tissue calcification. The co-existence of the two diseases is probably incidental.[ncbi.nlm.nih.gov]
  • Abstract Soft tissue calcification is sometimes found in scleroderma and dermatomyositis, however, to our knowledge, only 29 cases of systemic lupus erythematosus (SLE) were reported to have soft tissue calcification.[jstage.jst.go.jp]
  • Soft tissue calcification, with special reference to its occurrence in “collagen disease”. Ann. int. Med. 36 , 1050. PubMed Google Scholar Copyright information Dr. K C Chaudhuri Foundation 1968 Authors and Affiliations A. S. Sethi 1 S. C.[link.springer.com]
  • Soft tissue calcifications may be easily demonstrated on plain radiographs, but CT scan is more effective in demonstrating the location and extent of calcifications.[sajr.org.za]
  • This paper presents a review of the literature and adds a new case of calcinosis universalis and its evolution in 28 months, describing laboratory and radiograph findings and suggesting the differential diagnosis among processes of soft tissue calcification[ncbi.nlm.nih.gov]
Right Pleural Effusion
  • Her chest radiograph showed a right pleural effusion with cardiomegaly (Fig. 1), and radiographs of the hips and hands demonstrated widespread calcifications in the soft tissue and no evidence of arthropathy (Figs 2a and b).[sajr.org.za]
Microcytosis
  • The laboratory tests showed microcytosis, serum calcium 5.3 mEq/L (n.v. 4.5-5.5), phosphoremia 6.06 mg/dl (n.v. 4-6, 5), parathyroid hormone 64.05 pg/ml (n.v. 7-83 ), urinary phosphorus 0.12 g/24h (n.v. 0.5-0.8), tubular reabsorption of phosphate 85%[ejpd.com]

Treatment

  • Different treatments have been used in an attempt to clear calcinosis lesions and prevent its recurrence but none has been clearly effective.[ncbi.nlm.nih.gov]
  • The treatment and possible mechanisms of this complication are discussed.[ncbi.nlm.nih.gov]
  • Medical and surgical treatment are options to cure calcinosis cutis. Medical treatment using bisphosphonate. A better understanding of the process of calcinosis cutis will lead to therapies to improve patient morbidity.[ncbi.nlm.nih.gov]

Prognosis

  • Treatment and prognosis No established therapy is recognized, but calcium chelates (EDTA) bisphosphonates, and steroids have been used with little consistent benefit.[radiopaedia.org]
  • The prognosis however is generally poor. References 1. Black MM, Gawkrodyer DJ, Seymour CA, Weismann K. Metabolic and nutritional disorders. In: Champion RH, Burton JL, Ebling FJG, eds. Oxford: Blackwell Scientific Publication, 1993:2295-381. 2.[ijdvl.com]
  • Chester V Oddis, Idiopathic inflammatory myopathy: management and prognosis, Rheumatic Disease Clinics of North America, 28, 4, (979), (2002). Ann M.[doi.org]
  • In general, dysphagia portends a poor prognosis. Vascular involvement in DM usually presents as small vessel vasculitis. 1,8 Treatment Treatment of DM. DM usually responds well to treatment with systemic steroids.[woundsresearch.com]

Etiology

  • Abstract Calcinosis universalis is a rare and interesting condition of unknown etiology characterized by deposits of calcium in the skin and subcutaneous tissues. The diagnosis is easily made roentgenographically.[pubs.rsna.org]
  • Since then, the syndrome has been the subject of a great deal of discussion and controversy as to the etiology, the pathology and the therapy concerned.[jamanetwork.com]
  • The etiology is not known.[nejm.org]

Epidemiology

  • 2:1, and which has an incidence of 3.2/1,000,000 children and adolescents/year in the United States. 7 Despite its rarity, it is the fourth most frequent disease at tertiary pediatric rheumatology services, and there are no Brazilian studies of its epidemiology[jped.com.br]
Sex distribution
Age distribution

Pathophysiology

  • Little is known about the pathophysiology of calcinosis and there is no universally recognized treatment. Intravenous pamidronate significantly reduced the calcinosis. Early recognition and aggressive treatment of calcinosis is highlighted.[content.iospress.com]
  • […] scleroderma CREST syndrome, lupus erythematosus, overlap syndromes or dermatomyosistis. 1 Likewise the rarely described calcinosis cutis universalis is mostly associated with collagenoses but even other diseases like graft-versus-host can be rare causes. 2 Pathophysiologically[academic.oup.com]
  • […] subcutaneous fatty tissue septa, intramuscular septa, muscle fasciae, tenosinovitis or articular sinovitis. [4] Inflammatory stage of morphea reveal infiltration in the sucutaneous tissue with increase signal intensity on STIR sequences. [5] Depending on the pathophysiologic[mjdrdypu.org]

Prevention

  • Different treatments have been used in an attempt to clear calcinosis lesions and prevent its recurrence but none has been clearly effective.[ncbi.nlm.nih.gov]
  • Succinct, targeted coverage of normal childhood growth and development, as well as the diagnosis, management, and prevention of common pediatric diseases and disorders, make this an ideal medical reference book for students, pediatric residents, nurse[books.google.com]
  • Although the existing lesions showed slow improvement, systemic pamidronate therapy was effective in preventing the occurrence of new lesions. Surgical excision proved to be an effective and successful treatment.[karger.com]
  • Your skin Holds body fluids in, preventing dehydration Keeps harmful microbes out, preventing infections Helps you feel things like heat, cold, and pain Keeps your body temperature even Makes vitamin D when the sun shines on it Anything that irritates[icdlist.com]
  • Early diagnosis of the disease and effective treatment are essential to prevent calcinosis universalis from complicating this disease in children.[jle.com]

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