Four new cases of stiff skin syndrome with unusual presentations

Deng, Xiuyuan; Chen, F.; Song, Zhiqiang; Yan, Hong‐Bin; You, Yi; Zhong, Baiyu; Yang, Xichuan; Fei, Hao

doi:10.1111/jdv.12657

Cited by 10 publications

(9 citation statements)

References 5 publications

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“…In many cases, chest expansion is decreased due to the decreased elasticity and increased thickening of the skin in the thoracic region, causing restrictive changes in the lungs. [1][2][3][4][5][6][7] It is not a progressive disorder; 1-8 however, two cases reportedly progressed from segmental SSS to widespread SSS. 7,9 Therapeutic exercises and rehabilitation programs are suggested for preventing joint contractures.…”

mentioning

confidence: 99%

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Middle-Aged Female Diagnosed With Widespread Stiff Skin Syndrome

Sarı

Çirakoglu

2018

Arch Rheumatol

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mentioning

confidence: 99%

“…Corticosteroids and other immunosuppressants have proven ineffective. [1][2][3][4][5][6][7][8][9] Only mycophenolate mofetil resolved skin lesions and improved joint ROM in two cases. 10 Our patient underwent active rehabilitation with regularly performing ROM exercises and stretching exercises for alleviating contracture.…”

mentioning

confidence: 99%

Middle-Aged Female Diagnosed With Widespread Stiff Skin Syndrome

Sarı

Çirakoglu

2018

Arch Rheumatol

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“…1-3 However, bilateral involvement and pigmentary changes have also been reported. 6 A peau d'orange appearance was characteristic; it was present in all our patients, and could be identified in most published clinical pictures. [1][2][3]5 Despite being less frequent and severe than in the widespread version, the decreased joint mobility and leg discrepancy, found in Patients 3 and 1, respectively, have also been reported previously in SSSS.…”

Section: Reportmentioning

confidence: 54%

“…The histological findings were in agreement with previously reported features. 3,6 The increased dermal mucin might result in the decreased echogenicity in the dermis identified in HFUS. The lack of inflammatory infiltrates may explain the absence of pigmentary changes.…”

Section: Reportmentioning

confidence: 99%

Clinical presentation, sonographic features and treatment options of segmental stiff skin syndrome

Quintana‐Castanedo¹,

Bandera²,

Rodríguez³

et al. 2020

Clin Experimental Derm

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Segmental stiff skin syndrome is a rare genetic connective tissue disease, which is often misdiagnosed. High-frequency ultrasonography can represent a useful clinical adjunct in the differential diagnosis of this condition, in conjunction with the clinical and histopathological findings. Treatment options are limited and evidence is scarce. We present the clinical, sonographic and histological features of five paediatric patients diagnosed at our institution and discuss their response to treatment.

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“…Familiar hereditary subtype is caused by heterozygosity for a mutation in the gene that encodes fibrillin-1 (FBN1). Dysfunction of this gene results in the production of giant collagen fibrils in the affected fascia [50].…”

Section: Stiff Skin Syndromementioning

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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Four new cases of stiff skin syndrome with unusual presentations

Abstract: 7 Vates P, Jones S. Heparin skin necrosis-an important indicator of potentially fatal heparin hypersensitivity.

Cited by 10 publications

References 5 publications

Middle-Aged Female Diagnosed With Widespread Stiff Skin Syndrome

Middle-Aged Female Diagnosed With Widespread Stiff Skin Syndrome

Clinical presentation, sonographic features and treatment options of segmental stiff skin syndrome

Systemic Sclerosis Mimics

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