What Is Nodular-Keloidal Scleroderma?

Keloidal scleroderma: Case report and review

“…Ten percent of cases noted an external trigger prior to the onset of keloidal plaques, including infection, D-penicillamine, tetanus vaccine, and environmental exposures. One patient, who already had a diagnosis of keloidal scleroderma, did not have any involvement at a recent surgical site [1]. Laboratory values demonstrated the majority of patients were ANA positive (63%) and only 10% demonstrated anti-SCL-70 positivity.…”

“…Although there have been some reports suggesting that keloidal scleroderma may represent two distinct processes with keloid formation causally unrelated to sclerosis, others suggest that there is a combined mechanism with a dermal inflammatory process of sclerosis forming keloidal lesions [1]. High levels of tenascin have been histologically observed in keloidal scleroderma lesions as well as increased levels of TFG-beta cytokines [1,2]. Tenascin has been shown to have distinct mid-dermal distribution in sclerodermal lesions, reflecting active fibrosis [1].…”

“…High levels of tenascin have been histologically observed in keloidal scleroderma lesions as well as increased levels of TFG-beta cytokines [1,2]. Tenascin has been shown to have distinct mid-dermal distribution in sclerodermal lesions, reflecting active fibrosis [1]. A strikingly different tenascin distribution in the nodular lesions of a single case as compared to sclerotic tissue has been shown, suggesting a differing pathological course between the nodular and sclerotic lesions [1].…”

“…Tenascin has been shown to have distinct mid-dermal distribution in sclerodermal lesions, reflecting active fibrosis [1]. A strikingly different tenascin distribution in the nodular lesions of a single case as compared to sclerotic tissue has been shown, suggesting a differing pathological course between the nodular and sclerotic lesions [1]. However, keloidal scleroderma lesions have shown increased levels of TGF-beta and connective tissue growth factor (CTF), which is similarly seen in fibroblasts of classic sclerodermal lesions suggesting analogous pathogenesis of collagen synthesis [1,2].…”

“…The majority of patients presented with sclerodactyly as well as extracutaneous manifestations of systemic scleroderma (Table 1). Pulmonary involvement was reported in 28% and renal involvement in 5% [1]. Ten percent of cases noted an external trigger prior to the onset of keloidal plaques, including infection, D-penicillamine, tetanus vaccine, and environmental exposures.…”