In situ immunophenotype of the inflammatory infiltrate in eosinophilic fasciitis.
OBJECTIVE
Eosinophilic fasciitis (EF) is histologically characterized by a fibrous and inflammatory thickening of subcutaneous septal-fascial-perimysial collagenous scaffold. This study aims to define the immunophenotype of inflammatory cells of fascia and muscle underlying the in situ immune response in EF.


METHODS
In 11 cases of EF, we determined the phenotype of inflammatory cells, expression of MHC class I and class II antigens, and C5b9 membranolytic attack complex (MAC) deposits by immunohistochemistry analysis of fascia tissue. Muscle biopsies from 9 patients with active dermatomyositis and 5 with active polymyositis were used as controls. Results. In all patients but one, the inflammatory infiltrate was mainly composed of macrophages associated with CD8+ T lymphocytes (CD4/CD8 ratio < 1) and few eosinophils. Cytotoxic properties were found in 14% of CD8+ T lymphocytes, as shown by granzyme B expression. MHC Class I antigens were overexpressed (5/7) by muscle fibers, with a paratrabecular reinforcement in 4 cases. MHC class II antigens were not expressed by muscle fibers except in one case. C5b9 MAC deposits were not detected.


CONCLUSIONS
Our in situ characterization of inflammatory infiltrate demonstrates the predominancy of macrophages and CD8+ T lymphocytes. Some of these CD8+ lymphocytes contain granzyme B, thus suggesting a cytotoxic cellular immune response in EF, which could be triggered by infectious or environmental agents.
Toquet
Claire
C
Department of Pathology, Internal Medicine, INSERM U539, Nantes, France. claire.toquet@chu-nantes.fr
Hamidou
Mohamed Amine
MA
Renaudin
Karine
K
Jarry
Anne
A
Foulc
Phryné
P
Barbarot
Sébastien
S
Laboisse
Christian
C
Mussini
Jean-Marie Gilbert
JM
D016516:CD4-CD8 Ratio; D018414:CD8-Positive T-Lymphocytes / Q000276:immunology / Q000378:metabolism; D004804:Eosinophils / Q000276:immunology*; D005208:Fasciitis / Q000276:immunology* / Q000473:pathology*; D053804:Granzymes; D006801:Humans; D016130:Immunophenotyping / Q000379:methods*; D008264:Macrophages / Q000276:immunology; D018485:Muscle Fibers, Skeletal / Q000276:immunology / Q000473:pathology; D012697:Serine Endopeptidases / Q000378:metabolism
C509213:GZMB protein, human; D053804:Granzymes; D012697:Serine Endopeptidases