eMedicine Specialties > Rheumatology > Systemic Rheumatic Disease
Eosinophilic Fasciitis: Differential Diagnoses & Workup
Updated: Oct 2, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Differential Diagnoses
Eosinophilia
Eosinophilia-Myalgia Syndrome
Localized Fibrosing Disorders - Linear
Scleroderma, Morphea, and Regional Fibrosis
Scleroderma
Systemic Sclerosis
Toxic Oil Syndrome
Other Problems to Be Considered
Differential considerations include the following:
- The localized forms of scleroderma, morphea, and linear forms of scleroderma
- Limited and diffuse cutaneous systemic sclerosis
- Other localized cutaneous fibrosing disorders, eg, nephrogenic systemic fibrosis, scleromyxedema, and scleredema
In general, these alternative etiologies can be excluded by the absence of peripheral eosinophilia. Additionally, the absences of Raynaud phenomenon, abnormal capillaroscopy findings, and visceral involvement are key findings that differentiate eosinophilic fasciitis (EF) from systemic sclerosis.
Eosinophilia-myalgia syndrome and toxic oil syndrome are two disorders that share common clinical and histopathological features with eosinophilic fasciitis, including peripheral eosinophilia. In contrast to eosinophilic fasciitis, these two conditions present in epidemic form and, after epidemiological analysis, appear to be almost universally toxin-associated.32,33
In 1989, an epidemic of a connective-tissue disease with peripheral eosinophilia and prominent myalgias was recognized in the United States and was therefore termed eosinophilic myalgia syndrome. Subsequent epidemiologic studies indicated that most individuals who developed eosinophilic myalgia syndrome had consumed l-tryptophan from a single source. Diffuse induration of the integument affecting the extremities and occasionally the torso, but sparing the face, hands, and feet, developed in a large number of patients with eosinophilic myalgia syndrome. In contrast to eosinophilic fasciitis, patients with eosinophilic myalgia syndrome typically presented with prominent systemic symptoms, including fever, myalgia, and rash.
Similarly, in 1980 in Spain, an epidemic of connective-tissue disease with peripheral eosinophilia was recognized. The epidemic was eventually traced to rapeseed oil that had been denatured with aniline and sold in bulk as olive oil for cooking. As many as 20,000 Spaniards were affected. Acute symptoms were similar to those of eosinophilic myalgia syndrome, with prominent systemic symptoms in addition to cutaneous changes. In contrast to eosinophilic myalgia syndrome, acute symptoms included more prominent pulmonary manifestations. In chronic disease, patients with toxic oil syndrome were more likely to demonstrate systemic (pulmonary, neurologic) symptoms.8,34,35
Workup
Laboratory Studies
Characteristic laboratory findings of eosinophilic fasciitis (EF) include the following:
- Peripheral blood eosinophilia is present in 61%-83% of patients. The degree of eosinophilia is variable over time, even in the absence of specific therapy.15,36,37
- Hypergammaglobulinemia is characteristic, although this finding varies widely by case series, occurring in 18%-67% of patients. It is most often due to a polyclonal increase in immunoglobulin G.16,17,36
- An increase in the erythrocyte sedimentation rate (ESR) is found in 29%-70% of cases.16
Additional laboratory findings of eosinophilic fasciitis include the following:15,17,36
- Serum creatine kinase and aldolase levels are generally normal.
- Rheumatoid factor (RF) and antinuclear antibodies are occasionally positive.
- Hematologic abnormalities and disease are associated with eosinophilic fasciitis. Aplastic anemia, although rare, is the most frequent common associated hematological complication, but cases have been described with thrombocytopenia, hemolytic anemia, pernicious anemia, lymphoma, and leukemia.38,39
- Borrelia serology or polymerase chain reaction (PCR) findings are occasionally positive and may suggest a treatable etiology. However, as discussed above, the exact correlation between eosinophilic fasciitis and Borrelia remains unclear.23,24
- Metalloproteinase 1 (TIMP-1) may be a new serological marker of disease activity.9
Imaging Studies
- MRI is the imaging modality of choice. MRI of the involved areas shows characteristic findings of fascial thickening, abnormal signal intensity, and contrast enhancement. Additionally, MRI aids in making the diagnosis, locating the biopsy site, and monitoring the response to treatment.40,41,42,43
- Although it has not been used frequently or studied extensively in eosinophilic fasciitis, one case report has shown that ultrasonography can aid in early diagnosis.44
Other Tests
- While eosinophilic fasciitis is generally not associated with myositis or myopathy, electromyography has occasionally been performed, and findings may be abnormal in the presence of normal serum muscle enzymes.17
- Pulmonary function testing may show a restrictive pattern in patients with severe truncal involvement.17
Procedures
- Definitive diagnosis relies on a full-thickness incisional skin biopsy. The specimen should include the skin, fat, fascia, and superficial muscle in continuity. Biopsy is especially important in an atypical presentation.45,46,47
Histologic Findings
Inflammation, edema, thickening, and sclerosis of the fascia are hallmarks of eosinophilic fasciitis. Acute findings include infiltration of deep fascia and an adjacent subcutis layer with lymphocytes, plasma cells, histiocytes, and eosinophils. Distribution of the eosinophils in the fascia may be focal, and a close relationship appears to exist between blood and tissue eosinophilia. In the deeper portions of the panniculus, a similar infiltrate is found in the fibrous septa and at the periphery of the fat lobules. Deep in the fascia, the inflammatory infiltrate can extend into the epimysium, perimysium, and endomysium. In addition, vascular cuffing with lymphocytes and plasma cells is often seen.7,11,48
As the disease progresses, inflammatory changes are replaced by generalized sclerosis and thickening of the fascia and adjacent tissue layers. The sclerosis can be dense with hyalinized collagen bands running parallel to the fascia and small foci of fat cells trapped between them.36,49
Eosinophilic fasciitis. Top: In this gross specimen, the dermis (A), subcutaneous adipose tissue (B), and skeletal muscle do not appear unusual. However, the fascia (D) is markedly thickened. Bottom left: The gross findings are recapitulated in this low-power photomicrograph. The epidermis, dermis (A), and subcutaneous adipose tissue are not remarkable in this case. The fascia (D) is markedly thickened and focally infiltrated by inflammatory cells (E). The small amount of skeletal muscle (C) appears normal (hematoxylin and eosin stain at low power). Bottom right: A close-up photograph of a portion of the fascia showing mostly edematous cellular connective tissue (F). It is focally infiltrated by inflammatory cells, including lymphocytes, plasma cells, and histiocytes. The more intensely stained hypocellular pink bands across the top of the field (G) are part of an interstitial exudate of fibrin (hematoxylin and eosin stain at medium power).
More on Eosinophilic Fasciitis |
| Overview: Eosinophilic Fasciitis |
Differential Diagnoses & Workup: Eosinophilic Fasciitis |
| Treatment & Medication: Eosinophilic Fasciitis |
| Follow-up: Eosinophilic Fasciitis |
| Multimedia: Eosinophilic Fasciitis |
| References |
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Further Reading
Keywords
eosinophilic fasciitis, EF, eosinophilia, scleroderma, systemic sclerosis, elevated sedimentation rate, elevated ESR, hypergammaglobulinemia, fascial thickening, hematologic disease, Shulman syndrome, Shulman's syndrome, peripheral eosinophilia, fasciitis, fibrosing disorders, diffuse fasciitis with eosinophilia


Differential Diagnoses & Workup: Eosinophilic Fasciitis