Histological features of localized scleroderma ‘en coup de sabre’: a study of 16 cases

Abstract

Vacuolar degeneration at the dermoepidermal junction is a common histological feature in ECDS and perivascular and/or periappendageal lymphocytic infiltrate and vacuolar degeneration of follicular epithelium are characteristic especially in early ECDS, further supporting a canonical idea that the elimination of mutated epidermal cells by immune surveillance contributes to tissue damage and resultant fibrosis in localized scleroderma.

Serum levels of genomic DNA of α1(I) collagen are elevated in scleroderma patients

“…Serum COL1A1 gDNA levels were slightly higher in morphea patients than healthy control subjects, which may be associated with the notion that endothelial cell abnormality is also seen in localized scleroderma . Although there was no statistically significant difference, the levels in SSc patients tended to be higher than those in morphea patients.…”

“…Serum COL1A1 gDNA levels were slightly higher in morphea patients than healthy control subjects, which may be associated with the notion that endothelial cell abnormality is also seen in localized scleroderma. 12 Although there was no statistically significant difference, the levels in SSc patients tended to be higher than those in morphea patients. This tendency may be consistent with the severity of vascular endothelial cell damage in these diseases, because vasculopathy is usually more frequent in SSc patients than morphea patients.…”

Linear Morphea Mimicking Lichen Striatus in Its Early Presentation

“…Vacuolar changes with necrotic keratinocytes and melanin incontinence can be found. In late linear morphea, the predominant feature is epidermal atrophy and dermal fibrosis (16). In lichen striatus, the same interface dermatitis pattern can be seen, with a lichenoid infiltrate in the papillary dermis and along adnexal structures.…”

Scleroderma en coup de sabre with recurrent episodes of brain hemorrhage

“…6 Possibly related to this developmental process, previous publications have reported the comorbidity of intracranial abnormalities in the cases of ECDS. 5 According to their report, the patient's neurological symptoms were ameliorated by immunosuppressive agents, suggesting that there may be inflammatory or immunemediated mechanisms underlying the associated cerebral abnormalities in ECDS. A published work review by Chiu et al 3 revealed that among 51 patients with ECDS and Parry-Romberg syndrome, which is also a variant of linear scleroderma, up to 73% had intracranial abnormalities.…”

“…4 It is postulated that the linear and vertical destructive changes even to the depth of muscles or bones result from tissue damage by immune surveillance to eliminate cells with somatic mutations originally derived from a single somatically mutated embryonic cell which distribute along the Blaschko's lines in the developmental process. 5 On the scalp, a single Blaschko's line represents the distribution of cells derived from a single neural crest cell that has moved along the primitive streak. 5 On the scalp, a single Blaschko's line represents the distribution of cells derived from a single neural crest cell that has moved along the primitive streak.…”