Th1‐ and Th17‐polarized immune infiltrates in eosinophilic fasciitis—A potential marker for histopathologic distinction from morphea

• Published in: Journal of cutaneous pathology Vol. 44; no. 6; pp. 548 - 552
• Main Authors: Moy, Andrea Primiani, Maryamchik, Elena, Nikolskaia, Olga V., Nazarian, Rosalynn M.
• Format: Journal Article
• Language: English
• Published: Oxford, UK Blackwell Publishing Ltd 01-06-2017; Wiley Subscription Services, Inc
• Subjects: Adult; Aged; Aged, 80 and over; Antigens, Differentiation - immunology; CD3 antigen; CD4 antigen; CD8 antigen; Eosinophilia - immunology; Eosinophilia - pathology; eosinophilic fasciitis; Fasciitis; Fasciitis - immunology; Fasciitis - pathology; Female; GATA-3 protein; Helper cells; helper T‐cell; Humans; immune; Immunohistochemistry; Immunoregulation; Leukocytes (eosinophilic); Lymphocytes T; Male; Middle Aged; morphea; Polarization; Retrospective Studies; Scleroderma; Scleroderma, Localized - immunology; Scleroderma, Localized - pathology; Stat3 protein; Th1 Cells - immunology; Th1 Cells - pathology; Th17; Th17 Cells - immunology; Th17 Cells - pathology; Transcription factors; morphea; eosinophilic fasciitis; immune; Th17; helper T-cell
• ISSN: 0303-6987; 1600-0560
• DOI: 10.1111/cup.12947

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.
Materials & 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.
Conclusion
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.