Middle-Aged Female Diagnosed With Widespread Stiff Skin Syndrome

Sarı, Aylin; Çirakoglu, Derya

doi:10.5606/archrheumatol.2018.6805

Cited by 2 publications

(2 citation statements)

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“…Segmental stiff-skin syndrome is segmentally distributed with unilateral body affectation, has female predominance in contrast to the widespread stiff-skin syndrome, which has no gender predominance and less clinical consequences. 13,14 The clinical findings in our patient were consistent with segmental stiff skin syndrome. This is evidenced by the unilateral involvement of the lower extremities, gluteal and trunk muscles, and limited mobility of the left hip joint.…”

Section: Discussionsupporting

confidence: 74%

“…In addition, some investigators have found high levels of pro-inflammatory markers such as interleukin-6, transforming growth factor-2 and tumor necrosis factor-alpha, suggesting an inflammatory pathogenesis. 7,11,13,14 Symptoms of stiff skin syndrome include a rock-hard deep skin hardening that is inherently connected to the underlying tissue, reduced joint mobility, and hypertrichosis. The buttocks, thigh and shoulder are the most affected body regions, but ocular involvement has been reported in rare cases.…”

Section: Discussionmentioning

confidence: 99%

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Case Report: A case of stiff skin syndrome from a rural tertiary hospital in Eastern Cape

et al. 2022

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We present a case report of a four-year-old girl with stiff skin syndrome associated with bone involvement at the femoral head. She presented with thickness of the skin of the trunk and limbs, noted from the age of one year. This was associated with hypertrichosis and reduced joint mobility of the left lower extremity. The patient received physiotherapeutic treatments and underwent orthopedic examinations. A diagnosis of stiff skin syndrome should be considered in infants and children with thick skin, particularly if there is associated hypertrichosis. Important differentials to rule out include generalized morphea and systemic sclerosis. In this piece, we share insight on how a young girl with stiff skin syndrome associated with bone involvement at the femoral head presented and was managed in a rural tertiary health facility despite resource constraints.

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Section: Discussionsupporting

confidence: 74%