Eosinophilic fasciitis in Adult
The disease is very rare, with only about 250 cases reported in the medical literature. Individuals of Northern European descent have been most often reported to suffer from EF. The disease has occurred in patients of all ages, from childhood to the elderly, but most patients are middle aged.
Classically, EF presents with symmetrical edema and aching of the affected extremities, which progress rapidly to fibrosis, induration, and a peau d'orange appearance. The skin in EF is often bound down, and this accentuates the natural planes between muscles, giving a muscular or grooved appearance. Subacute and more insidious forms of EF also exist. In about one-quarter of patients, trauma or intense exercise precedes the onset of the disease.
While some authorities believe that EF overlaps with deep morphea and scleroderma, features that distinguish EF are a peripheral eosinophilia (in 60% to 80% of patients), hypergammaglobulinemia (in 20% to 70% of patients), and an absence of Raynaud phenomenon, common to scleroderma. In a retrospective study, 21 of 60 patients (35%) had concurrent plaque morphea.
Immune-mediated anemia, thrombocytopenia, pure red cell aplasia, aplastic anemia, myelodysplastic syndromes, and lymphoproliferative processes (T- or B-cell lymphoma, multiple myeloma) have occurred concomitantly in few reported cases of EF.
For more information, see OMIM.
M35.4 – Diffuse (eosinophilic) fasciitis
24129002 – Fasciitis with eosinophilia syndrome
- Scleroderma – a more superficial fibrotic connective tissue disease that is associated with Raynaud phenomenon and internal visceral involvement, such as esophageal dysfunction
- Morphea – a form of cutaneously limited scleroderma characterized by isolated plaques of the skin; it is usually not as symmetrical, extensive or abrupt as EF
- Scleromyxedema – presents with papules coalescing into indurated plaques on the extremities and is associated with paraproteinemia as well, but biopsy shows a hypercellular and myxedematous superficial fibrosis rather than a deep fibrosis of the fascia
- Nephrogenic systemic fibrosis – occurs in the setting of significant kidney disease and is highly associated with the administration of gadolinium-based contrast agents during periods of renal insufficiency
- Eosinophilia-myalgia syndrome / toxic oil syndrome – in the 1980s and early 1990s, contaminated rapeseed oil and contaminated tryptophan supplements resulted in a disease presentation similar to EF that caused erythema, edema, fibrosis, and joint contractures, but the illnesses disappeared with identification of the contaminated ingestants