Stiff skin syndrome

Geng, Shuang; Xb, Lei; Toyohara, J. P.; Zhan, Pingyu; Wang, J; Tan, Sanda

doi:10.1111/j.1468-3083.2006.01619.x

Cited by 25 publications

(16 citation statements)

References 9 publications

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“…The appearance of the disease in early childhood, the limitation of joint mobility, the proximal rather than a distal stiffness and the absence of organ involvement are the main clinical findings that differentiate SSS from SSc [7,17]. Moreover, microvascular abnormalities, RP and autoantibodies were absent in SSS [18]. However, in our SSS patient, a loss of microvessels was clearly evident at the dermal-epidermal junction and in the papillary dermis, despite the overexpression of VEGF found in the dermis.…”

Section: Discussionmentioning

confidence: 99%

Stiff skin syndrome: evidence for an inflammation-independent fibrosis?

Guiducci¹,

Distler²,

Milia³

et al. 2009

Rheumatology

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Section: Discussionmentioning

confidence: 99%

Stiff skin syndrome: evidence for an inflammation-independent fibrosis?

Guiducci¹,

Distler²,

Milia³

et al. 2009

Rheumatology

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“…The condition is stable or slowly progressive, and abnormalities of internal organs are not typically observed. In differential diagnosis the disease may resemble scleredema, deep morphea, or linear scleroderma [51].…”

Section: Clinical Featuresmentioning

confidence: 99%

“…Treatment of stiff skin syndrome is very difficult, and no effective treatments have been reported. Physical therapy and regimen measures for the patient can help to prevent progressive joint contractures and immobility [51,52].…”

Section: Treatmentmentioning

confidence: 99%

Systemic Sclerosis Mimics

Kodet¹,

Oreská²

2019

New Insights Into Systemic Sclerosis [Working Title]

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Many clinical conditions are presenting with sclerosis of the skin and with tissue fibrosis. These conditions may be confused with systemic sclerosis (SSc, scleroderma). These diseases and disabilities are generally referred to as systemic sclerosis mimics or scleroderma-like syndromes. These disorders have very different etiologies and often an unclear pathogenetic mechanism. Distinct clinical characteristics, skin histology, and disease associations may allow distinguishing these conditions from systemic sclerosis and from each other. A histopathological examination with clinicopathological correlation for diagnosis is important to spare the patients from ineffective treatments and inadequate management. In this chapter, we discussed localized scleroderma, lichen sclerosus, nephrogenic systemic fibrosis, eosinophilic fasciitis, scleromyxedema, and scleredema. These are often detected in the primary care setting and referred to rheumatologists for further evaluation. Rheumatologists, or preferably in collaboration with a dermatologist, must be able to promptly recognize them to provide valuable prognostic information and appropriate treatment options for affected patients.

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“…In exceptionally severe cases, nearly generalized induration of the trunk and proximal extremities can be seen. Although rare, the incidence of the disease is apparently worldwide, as afflicted children have been diagnosed in the United States, Europe, China, 5 Turkey, 6 and Thailand (unpublished observation).…”

mentioning

confidence: 96%