title: Th1- and Th17-polarized immune infiltrates in eosinophilic fasciitis-A potential marker for histopathologic distinction from morphea.
abstract: BACKGROUND
  Morphea (localized scleroderma) and eosinophilic fasciitis (EF) are rare fibrosing disorders which may present a diagnostic challenge. While histopathologic features are often distinct, in some cases there may be overlap. T-cells contribute to etiopathogenesis of both autoimmune conditions. We sought to determine whether T-cell immune polarization enables histopathologic distinction.
  
  
  METHODS
  We retrospectively examined clinicopathologically confirmed cases of morphea (n = 12) and EF (n = 8) using immunohistochemistry for CD3, CD8, and dual staining for CD4 with T-bet, GATA-3, STAT-3 or BNC-2 (transcription factors reported to be specific and mutually exclusive for Th1, Th2, Th17 and Th22 cells, respectively) to characterize the T-cell infiltrate.
  
  
  RESULTS
  No significant difference in CD3+ cells was identified (P = .195), however, the CD4/CD8+ T-cell ratio was significantly greater in morphea compared to EF (1.2 and 0.6, respectively; P = .034). Th1/Th2 was significantly lower in morphea compared to EF (1.7 and 2.7, respectively; P = .027). The percent of Th17+ cells was significantly higher in EF (P = 0.041). No significant difference in percent of Th22+ cells was identified.
  
  
  CONCLUSIONS
  Morphea and EF may be histopathologically distinguished based on helper T-cell subtype polarization. These findings offer novel insight into our understanding of disease pathogenesis and support a role for Th1/Th2 immune regulation and Th17 inhibition in anti-fibrotic therapeutic strategy.
authors.affiliation: Pathology Service, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts.
authors.forename: Andrea Primiani
authors.initials: AP
authors.lastname: Moy
authors.affiliation: Pathology Service, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts.
authors.forename: Elena
authors.initials: E
authors.lastname: Maryamchik
authors.affiliation: Pathology Service, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts.
authors.forename: Olga V
authors.initials: OV
authors.lastname: Nikolskaia
authors.affiliation: Pathology Service, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts.
authors.forename: Rosalynn M
authors.identifier: http://orcid.org/0000-0003-4003-7193
authors.initials: RM
authors.lastname: Nazarian
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000943:Antigens, Differentiation / Q000276:immunology*; D004802:Eosinophilia / Q000276:immunology / Q000473:pathology; D005208:Fasciitis / Q000276:immunology / Q000473:pathology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012594:Scleroderma, Localized / Q000276:immunology / Q000473:pathology; D018417:Th1 Cells / Q000276:immunology / Q000473:pathology; D058504:Th17 Cells / Q000276:immunology / Q000473:pathology
chemical_list: D000943:Antigens, Differentiation
keywords: Th17; eosinophilic fasciitis; helper T-cell; immune; morphea