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![Skin biopsy revealed thickened collagen, minimal inflammatory infiltrate, well‐preserved appendages, and adipocyte entrapment in dermis (hematoxylin and eosin [HE] staining, original magnification, ×40)](publication/361230065/figure/fig2/AS:11431281173617227@1688992240642/Skin-biopsy-revealed-thickened-collagen-minimal-inflammatory-infiltrate-well-preserved.png)
Skin biopsy revealed thickened collagen, minimal inflammatory infiltrate, well‐preserved appendages, and adipocyte entrapment in dermis (hematoxylin and eosin [HE] staining, original magnification, ×40)
Source publication
Stiff Skin Syndrome (SSS) is a rare disorder characterized by skin induration and limited joint mobility in the absence of visceral, musculoskeletal, vascular, or immunologic abnormalities. Distinctive subsets of SSS could be distinguished by various manifestation and mechanism, which accounts for the high heterogeneity in SSS cases. Although rehab...
Citations
Background
Stiff skin syndrome (SSS) is a rare disease characterized by thickened, indurated skin and limited joint movement. Multiple diverse phenotypes have been reported, and the correlation of severity with the clinical heterogeneity and histopathological findings of SSS needs to be refined.
Objective
To define subtypes based on clinical features and predict the prognosis of a new SSS classification.
Methods
Eighty-three patients with SSS were retrospectively reviewed for clinicopathological manifestations and routine laboratory workup, including 59 cases obtained from a PubMed search between 1971 and 2022 and 24 cases diagnosed in our department between 2003 and 2022.
Results
Among the 83 patients, 27.7, 41, and 31.3% had classic widespread, generalized segmental, and localized SSS, respectively. Joint immobility was present in 100, 71, and 20% of classic, generalized, and localized cases, respectively. Histopathologic findings were common among the 3 groups, and based on that, we further found a difference in the distribution of proliferative collagen. 54.5% of classic and 50% of generalized cases occurred throughout the dermis or the subcutis, whereas 76% of localized cases were mainly involved in the reticular dermis or subcutis. In patients with incipient localized SSS, 42% (21/50) developed generalized SSS, and only 6% (3/50) progressed to classic SSS, whereas more than half of the incipient generalized SSS cases (60.6%, 20/33) developed classic SSS.
Limitations
This retrospective study was limited to previously published cases with limited data.
Conclusions
We propose a distinct clinical classification characterized by lesion distribution, including classic widespread, generalized segmental, and localized SSS, associated with disease severity and prognosis.
Stiff skin syndrome (SSS), also known as congenital fascial dystrophy, is a rare disorder characterized by noninflammatory and progressive skin fibrosis, that may be misdiagnosed as scleroderma. Literature data on SSS is very limited, and there is no effective treatment. Herein, given its rarity, we report a 28-year old case of SSS to increase the awareness of clinicians about SSS and also to point out the potential role of intravenous immunoglobulin in such cases.
