Diffuse fasciitis with eosinophilia in childhood

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.