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Review
. 2008 Feb;34(1):199-220; ix.
doi: 10.1016/j.rdc.2007.11.001.

Scleroderma-like fibrosing disorders

Francesco Boin  1 , Laura K Hummers
Affiliations
Review

Scleroderma-like fibrosing disorders

Francesco Boin et al. Rheum Dis Clin North Am. 2008 Feb.

Abstract

Many conditions presenting with clinical hard skin and tissue fibrosis can be confused with systemic sclerosis (scleroderma). These disorders have very diverse etiologies and often an unclear pathogenetic mechanism. Distinct clinical characteristics, skin histology, and disease associations may allow one to distinguish these conditions from scleroderma and from each other. A prompt diagnosis is important to spare patients from ineffective treatments and inadequate management. This article highlights nephrogenic systemic fibrosis (nephrogenic fibrosing dermopathy), eosinophilic fasciitis (Shulman's syndrome), scleromyxedema, and scleredema. These often are detected in the primary care setting and referred to rheumatologists for further evaluation. Rheumatologists must be able to promptly recognize them to provide valuable prognostic information and appropriate treatment options for affected patients.

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Figures

Figure 1
Figure 1
Nephrogenic systemic fibrosis. (A) Dense dermal fibrosis is present under a normal epidermis. Disorganized collagen bundles are separated by large clefts and surrounded by numerous fibroblast-like (spindle) cells. Minimal perivascular inflammatory infiltrate is present. (B) The infiltrative process is deep, extending into subcutaneous adipose interlobular septa.
Figure 2
Figure 2
Eosinophilic fasciitis. (A) Patient presenting bilateral involvement of upper extremities with typical “woody” induration of the skin and “puckering”. (B) The same patient after 12 months of corticosteroids treatment.
Figure 3
Figure 3
Scleromyxedema. Patient with classic papular-waxy skin eruption of the face
Figure 4
Figure 4
Scleredema. Patient with diabetes mellitus type 2 and skin induration of the neck and upper back
See this image and copyright information in PMC

References

    1. Cowper SE, Robin HS, Steinberg SM, et al. Scleromyxoedema-like cutaneous diseases in renal-dialysis patients. Lancet. 2000;356(9234):1000–1. - PubMed
    1. Jan F, Segal JM, Dyer J, et al. Nephrogenic fibrosing dermopathy: two pediatric cases. J Pediatr. 2003;143(5):678–81. - PubMed
    1. Cowper SE. Nephrogenic Fibrosing Dermopathy NFD/NSF Website. 2001–2007. [Accessed 11/05/2007]. Available at http://www.icnfdr.org. - PubMed
    1. Cowper SE, Su LD, Bhawan J, et al. Nephrogenic fibrosing dermopathy. Am J Dermatopathol. 2001;23(5):383–93. - PubMed
    1. Ting WW, Stone MS, Madison KC, et al. Nephrogenic fibrosing dermopathy with systemic involvement. Arch Dermatol. 2003;139(7):903–6. - PubMed

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