eMedicine Specialties > Rheumatology > Systemic Rheumatic Disease
Eosinophilic Fasciitis
Updated: Nov 7, 2006
Introduction
Background
The early description of eosinophilic fasciitis (EF) by Shulman in 1975 was of a disorder characterized by peripheral eosinophilia and fasciitis, which could be differentiated from scleroderma by the distinctive pattern of skin involvement that spares the digits, involves fascia rather than dermis, and is not accompanied by Raynaud phenomenon.
In these early reports, the disorder was considered clinically and histopathologically localized to the fascia. Since 1974, more than 200 patients with eosinophilic fasciitis have been reported, and debates are still ongoing as to whether eosinophilic fasciitis represents a variant of scleroderma.
Several large reviews have generated a broader clinical image of the condition, but eosinophilia, elevated sedimentation rate, hypergammaglobulinemia, and fascial thickening are critical elements of the syndrome.
Visceral involvement in eosinophilic fasciitis is often mild and asymptomatic; however, an association with hematologic disease now is recognized and frequently carries a grave prognosis.
Pathophysiology
Although the etiology of eosinophilic fasciitis is unknown, recent studies have shed light on some of the mechanisms involved in its pathogenesis. Fibroblasts from lesional tissue of patients with eosinophilic fasciitis produce excess collagen in vitro and display elevated transforming growth factor-b (TGF-b) and type 1 collagen mRNA levels when examined via in situ hybridization with specific cDNA. Therefore, the pathogenesis appears to involve the concomitant increase in the expression of genes for TGF-b and extracellular matrix proteins in fibroblasts in the affected tissues.
One study showed that the fascial inflammatory infiltrate is predominantly composed of CD8 T lymphocytes, macrophages, and fewer eosinophils, suggesting a possible cytotoxic immune reaction in response to a possible infectious or environmental agent. Recent reports indicate that Borrelia burgdorferi may be a possible etiologic agent in some cases. Other studies showed elevated levels of serum manganese superoxide dismutase and tissue metalloproteinase 1 (TIMP-1), which may serve as a marker of disease activity.
Fasciitis may be a common manifestation of various pathophysiologic processes associated with eosinophilia. The existence of primary and secondary forms of fasciitis has recently been suggested.
Understanding the mechanisms involved in the development of fascial inflammation and fibrosis in these conditions may yield insights into the pathogenesis of other fibrotic skin diseases.
Some cases of eosinophilic fasciitis have been described as associated with ingestion of L-tryptophan (see Eosinophilia-Myalgia Syndrome [EMS] and Causes).
Frequency
United States
Eosinophilic fasciitis is very rare.
Mortality/Morbidity
No data are available on morbidity or mortality rates. Rarely, a fatal aplastic anemia may develop.
Race
Eosinophilic fasciitis affects whites more often than it affects blacks.
Sex
In adults, eosinophilic fasciitis predominantly affects women.
Age
The age range is 2-88 years, although most patients present during the third to sixth decades of life.
Clinical
History
- The onset of illness is not accompanied by fever or other systemic symptoms and, in about half of patients, follows an episode of strenuous physical exercise or activity.
- Swelling and progressive induration of the skin associated with aching of the extremities and occasional morning stiffness develop over a period of weeks. The distribution is most often in the upper extremity, proximal and distal to the elbow, and in the lower extremity, proximal and distal to the knee. Onset may be acute following some sort of strenuous exercise, or it may be subacute.
- Paresthesias may develop in a distribution pattern consistent with carpal tunnel syndrome, which has been reported.
- Inflammatory arthritis affects mainly the hands and wrists. Contractures occur in 75% of patients, typically affecting elbows, wrists, ankles, knees, hands, and shoulders.
- Neither Raynaud phenomenon nor symptoms of respiratory, gastrointestinal, or cardiac involvement are present.
Physical
- Upon examination, cutaneous induration with venous furrowing and irregular coarse puckering or fine dimpling (peau d'orange) is found. Skin changes affect all extremities in most cases, but they may be limited to the arms or abdomen.
- Other cutaneous changes reported include erythema, urticaria, bullae, alopecia, lichen sclerosis et atrophicus, vitiligo, and hyperpigmentation.
- Although any area of the body may be affected, arms, legs, hands, and feet are involved most commonly.
- A clawlike deformity of the hands has been described.
- Tenosynovitis of the flexor tendon sheath at the wrist causes carpal tunnel syndrome in 20% of the cases.
- Frequently, the inflammation extends deep from the fascia into the underlying muscle. This may be difficult to detect clinically.
- Clinically significant visceral involvement is rare, but specific testing may demonstrate subtle abnormalities similar to those found in localized scleroderma (morphea).
- Esophageal dysmotility has been reported rarely.
- Fasciitis of the thorax may result in restrictive ventilatory defects.
- Although cardiac involvement in the form of a pericardial effusion is seldom noted, it has been reported.
- Interestingly, Sjögren syndrome and hepatic and splenic enlargement also have been reported.
- Inflammatory arthritis occurs in 40% of patients, and joint contractures of the elbows, wrists, ankles, knees, and shoulders may develop in 55-75% of patients.
- A concurrent localized lesion of morphea may be seen in 25% of patients.
Causes
The etiology of eosinophilic fasciitis is not known, but it may follow strenuous activity. Some cases of eosinophilic fasciitis have been described to occur simultaneously with the appearance of EMS associated with L-tryptophan ingestion.More on Eosinophilic Fasciitis |
 Overview: Eosinophilic Fasciitis |
| Differential Diagnoses & Workup: Eosinophilic Fasciitis |
| Treatment & Medication: Eosinophilic Fasciitis |
| Follow-up: Eosinophilic Fasciitis |
| Multimedia: Eosinophilic Fasciitis |
| References |
| Next Page » |
References
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Kahari VM, Heino J, Niskanen L, et al. Eosinophilic fasciitis. Increased collagen production and type I procollagen messenger RNA levels in fibroblasts cultured from involved skin. Arch Dermatol. May 1990;126(5):613-7. [Medline].
Katz JD, Wakem CJ, Parke AL. L-tryptophan associated eosinophilia-myalgia syndrome. J Rheumatol. Nov 1990;17(11):1559-61. [Medline].
Lakhanpal S, Ginsburg WW, Michet CJ, et al. Eosinophilic fasciitis: clinical spectrum and therapeutic response in 52 cases. Semin Arthritis Rheum. May 1988;17(4):221-31. [Medline].
Lee P. Eosinophilic fasciitis: new associations and current perspectives [editorial]. J Rheumatol. Jan-Feb 1981;8(1):6-8. [Medline].
Peltonen J, Kahari L, Jaakkola S, et al. Evaluation of transforming growth factor beta and type I procollagen gene expression in fibrotic skin diseases by in situ hybridization. J Invest Dermatol. Mar 1990;94(3):365-71. [Medline].
Shulman LE. The eosinophilia-myalgia syndrome associated with ingestion of L- tryptophan. Arthritis Rheum. Jul 1990;33(7):913-7. [Medline].
Wollheim FA, Lindstrom CG, Eiken O. Eosinophilic fasciitis complicated by carpal tunnel syndrome. J Rheumatol. Sep-Oct 1981;8(5):856-60. [Medline].
Further Reading
Keywords
eosinophilic fasciitis, EF, eosinophilia, scleroderma, elevated sedimentation rate, elevated ESR, hypergammaglobulinemia, fascial thickening, hematologic disease, Shulman syndrome, Shulman's syndrome, peripheral eosinophilia, fasciitis, Borrelia burgdorferi, B burgdorferi
