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Diffuse fasciitis with eosinophilia in childhood
The Johns Hopkins Medical Journal
|November 1, 1982
Summary
Diffuse fasciitis with eosinophilia (DFE) is a connective tissue disease distinct from scleroderma and dermatomyositis. DFE presents with characteristic skin and fascial changes, eosinophilia, and responds well to steroid treatment.
Area of Science:
- Rheumatology
- Dermatology
- Pathology
Background:
- Diffuse fasciitis with eosinophilia (DFE) is a recently identified connective tissue disorder.
- It can be initially misdiagnosed as scleroderma or dermatomyositis.
- DFE primarily affects adults and involves extremities and trunk.
Observation:
- Clinical presentation includes firm, bound-down skin with puckering or ridging, leading to contractures within weeks.
- Peripheral blood may show eosinophilia and elevated immunoglobulins.
- Histological examination reveals characteristic fascial edema, thickening, and mononuclear cell infiltration.
Findings:
- DFE possesses distinct clinical and histological features differentiating it from scleroderma and dermatomyositis.
- Biopsies must include skin, subcutaneous tissue, fascia, and muscle as a single block for accurate diagnosis.
- The primary diagnostic finding is in the fascia, while other tissues remain unaffected.
Implications:
- DFE has a more favorable prognosis compared to scleroderma and dermatomyositis.
- Prompt diagnosis and treatment with corticosteroids lead to a favorable clinical response.
- Understanding DFE's unique characteristics is crucial for accurate diagnosis and management.