Dermatomyositis with Universal Calcinosis

Nielsen, Anni Brit Sternhagen; Johnson, E.; Hentzer, B; Kobayasi, T

doi:10.1111/j.1600-0560.1979.tb01175.x

Cited by 27 publications

(15 citation statements)

References 8 publications

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“…The slightly mineralized elastic fibers showed a completely different pattern of calcification as compared with the findings of Nielsen et al [3], who reported on deposits in the periphery of the elastic fiber. We found the nucléation of apatite crystals beginning around the microfibrils of the clastic fiber.…”

Section: Discussioncontrasting

confidence: 51%

“…According to Paegle [12] the calcium deposits are first situated in the ground sub stance, whereas Corneliuset al [ 13] found the earliest cal cium deposits developing within collagen fibrils and sub sequently extending into the ground substance when the apatite crystals are growing. Nielsen et al [3] studying a case of DCC universalis in dermatomyositis thought that the mineralized fibers surrounding the calcinotic areas were elastic ones. In contrast.…”

Section: Discussionmentioning

confidence: 99%

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Mineralization of Collagen and Elastic Fibers in Superficial Dystrophic Cutaneous Calcification: An Ultrastructural Study

Fartasch

Haneke²,

Hornstein

1990

Dermatology

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The ultrastructural morphology of localized skin calcifications without associated diseases and with normal serum calcium and phosphate ion values is still unknown. In a case of superficial dystrophic calcinosis cutis (DCC) the role of collagen elastin and ground substance in the process of calcification and the organization of the apatite crystals could be studied by light and electron microscopy despite technical difficulties in sectioning the hard tissue. Ultrastructural investigation revealed the nucleation of calcification being related to collagen and elastic fibers. No intracellular calcification was found. A flower-like arrangement of pleomorphic crystals was found around single collagen fibrils resembling the calcification of collagen seen in bone tissue. The elastic fibers showed a different pattern of calcification compared with other diseases (e.g. pseudoxanthoma elasticum) with known calcification of the elastic fibers. The process of mineralization was initially linked to the microfibrils of the elastic fiber.

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Section: Discussioncontrasting

confidence: 51%

Section: Discussionmentioning

confidence: 99%

Mineralization of Collagen and Elastic Fibers in Superficial Dystrophic Cutaneous Calcification: An Ultrastructural Study

Fartasch

Haneke²,

Hornstein

1990

Dermatology

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“…Nielsen and colleagues [10] stated that the accuracy of the selected area diffraction method used in their study was not sufficient to distinguish between hydroxyapatite and or fluoroapatite. The mineralized regions also contained small amounts of proteins that were significantly lower than those present in bone (Table 2), as determined in the FTIR-RM analyses.…”

Section: Discussionmentioning

confidence: 99%

Microstructure and mineral composition of dystrophic calcification associated with the idiopathic inflammatory myopathies

et al. 2009

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IntroductionCalcified deposits (CDs) in skin and muscles are common in juvenile dermatomyositis (DM), and less frequent in adult DM. Limited information exists about the microstructure and composition of these deposits, and no information is available on their elemental composition and contents, mineral density (MD) and stiffness. We determined the microstructure, chemical composition, MD and stiffness of CDs obtained from DM patients.MethodsSurgically-removed calcinosis specimens were analyzed with fourier transform infrared microspectroscopy in reflectance mode (FTIR-RM) to map their spatial distribution and composition, and with scanning electron microscopy/silicon drift detector energy dispersive X-ray spectrometry (SEM/SDD-EDS) to obtain elemental maps. X-ray diffraction (XRD) identified their mineral structure, X-ray micro-computed tomography (μCT) mapped their internal structure and 3D distribution, quantitative backscattered electron (qBSE) imaging assessed their morphology and MD, nanoindentation measured their stiffness, and polarized light microscopy (PLM) evaluated the organic matrix composition.ResultsSome specimens were composed of continuous carbonate apatite containing small amounts of proteins with a mineral to protein ratio much higher than in bone, and other specimens contained scattered agglomerates of various sizes with similar composition (FTIR-RM). Continuous or fragmented mineralization was present across the entire specimens (μCT). The apatite was much more crystallized than bone and dentin, and closer to enamel (XRD) and its calcium/phophorous ratios were close to stoichiometric hydroxyapatite (SEM/SDD-EDS). The deposits also contained magnesium and sodium (SEM/SDD-EDS). The MD (qBSE) was closer to enamel than bone and dentin, as was the stiffness (nanoindentation) in the larger dense patches. Large mineralized areas were typically devoid of collagen; however, collagen was noted in some regions within the mineral or margins (PLM). qBSE, FTIR-RM and SEM/SDD-EDS maps suggest that the mineral is deposited first in a fragmented pattern followed by a wave of mineralization that incorporates these particles. Calcinosis masses with shorter duration appeared to have islands of mineralization, whereas longstanding deposits were solidly mineralized.ConclusionsThe properties of the mineral present in the calcinosis masses are closest to that of enamel, while clearly differing from bone. Calcium and phosphate, normally present in affected tissues, may have precipitated as carbonate apatite due to local loss of mineralization inhibitors.

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“…The histopathology of surgically-removed specimens, often of longstanding duration, demonstrates chronic inflammatory cells encapsulating the mineral consisting apparently of a variety of cell types, including macrophages, giant cells, lymphocytes and eosinophils [33,56–58]. The calcium mineral itself may be a chemoattractant for macrophages and monocytes [59].…”

Section: Pathogenesismentioning

confidence: 99%

The Presentation, Assessment, Pathogenesis, and Treatment of Calcinosis in Juvenile Dermatomyositis

et al. 2014

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Calcinosis is one of the hallmark sequelae of juvenile dermatomyositis (JDM), and despite recent progress in the therapy of JDM, dystrophic calcification still occurs in approximately one-third of patients. This review discusses our current, albeit limited, understanding of risk factors for the development of calcinosis in JDM, as well as approaches to assessment, and current views on its pathogenesis. Anecdotal approaches to treating calcinosis associated with JDM, including both anti-inflammatory therapies and agents aimed at inhibiting the deposition of calcium hydroxyapatite, are reviewed. An improved understanding of the pathogenesis of calcinosis, the establishment of standardized measurement tools to assess calcinosis, and randomized controlled trials employing more sensitive outcome measures are needed to develop efficacious therapies for this often disabling complication.

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Dermatomyositis with Universal Calcinosis

Cited by 27 publications

References 8 publications

Mineralization of Collagen and Elastic Fibers in Superficial Dystrophic Cutaneous Calcification: An Ultrastructural Study

Mineralization of Collagen and Elastic Fibers in Superficial Dystrophic Cutaneous Calcification: An Ultrastructural Study

Microstructure and mineral composition of dystrophic calcification associated with the idiopathic inflammatory myopathies

The Presentation, Assessment, Pathogenesis, and Treatment of Calcinosis in Juvenile Dermatomyositis

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