Eosinophilic Fasciitis: Practice Essentials, Pathophysiology, Etiology (original) (raw)

Practice Essentials

Eosinophilic fasciitis (EF), also called Shulman syndrome, is a rare, localized fibrosing disorder of the fascia. [1] (See image below.) The etiology and pathophysiology are unclear.

Eosinophilic fasciitis. Top: In this gross specime

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).

In 1974, Shulman provided an early description of a disorder characterized by peripheral eosinophilia and fasciitis that 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. [2] In 1975, Rodnan et al proposed the term eosinophilic fasciitis for the disorder. [3] Eosinophilic fasciitis is relatively rare, with only about 300 cases reported; the literature consists of a relatively few large case series and multiple case reports. Therefore, the understanding of key aspects of the disease continues to evolve. [1]

The etiology of eosinophilic fasciitis remains unknown, although many possible triggers (in particular, sustained intense physical exercise) and disease associations have been suggested. Some aspects of pathophysiology have been elucidated; however, a more complete understanding has yet to develop.

The available literature has generated a broader clinical image of the condition, but fascial thickening in the setting of eosinophilia, elevated erythrocyte sedimentation rate, and hypergammaglobulinemia remain critical elements of the syndrome. Visceral involvement in eosinophilic fasciitis is generally absent, a finding that helps differentiate eosinophilic fasciitis from systemic sclerosis and other differential considerations. However, an association with several hematologic diseases is recognized and frequently carries a grave prognosis.

The diagnosis of eosinophilic fasciitis is suspected in a patient presenting with characteristic skin changes and consistent laboratory findings. It is confirmed with full-thickness biopsy or characteristic MRI findings. See Presentation and Workup.

Eosinophilic fasciitis is generally corticosteroid-responsive, and initial treatment regimens are based on this therapy. Multiple additional agents have been used in steroid-refractory disease. The evidence for many of these agents is anecdotal, and there is no general consensus regarding the best agent for treatment of steroid-resistant disease or cases refractory to steroid withdrawal. See Treatment and Medication.

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Pathophysiology

Although the etiology of eosinophilic fasciitis is unknown, studies have shed light on some of the mechanisms involved in its pathogenesis.

In general, the pathophysiology underlying eosinophilic fasciitis is postulated to involve an inflammatory response resulting in an activated inflammatory cell infiltrate of affected tissues and subsequent dysregulation of extracellular matrix production by lesional fibroblasts.

Viallard et al demonstrated that, when stimulated, peripheral blood mononuclear cells of eosinophilic fasciitis patients produce significantly higher amounts of five cytokines, including interleukin (IL)–5 and interferon (IFN)–gamma. [4] IL-5 is known to activate mature eosinophils and to stimulate eosinophil chemotaxis, growth, and differentiation. IFN-gamma activates tissue macrophages and T cells. The findings of Dziadzio et al support increased levels of IL-5 in eosinophilic fasciitis, in addition to increased levels of transforming growth factor (TGF)–beta, another fibrogenic cytokine. [5]

Toquet et al investigated the phenotype of the lesional inflammatory cell infiltrate in patients with eosinophilic fasciitis and demonstrated a predominance of macrophages, CD8+ lymphocytes, and few eosinophils. [6] Pathologic specimens from patients with eosinophilic fasciitis demonstrate increased numbers of eosinophils, especially early in the disease course.

Taken together, the findings of these studies suggest a mechanistic framework marked by a proinflammatory and fibrogenic cytokine response with resultant tissue inflammatory cell infiltration.

In the tissues, the end effector cell of fibrosis is the fibroblast. Fibroblasts from lesional tissue of patients with eosinophilic fasciitis produce excess collagen in vitro and display elevated TGF-beta and type 1 collagen mRNA levels when examined via in situ hybridization with specific cDNA. [7, 8] Therefore, the pathogenesis appears to involve the concomitant increase in the expression of genes for TGF-beta and extracellular matrix proteins in fibroblasts in the affected tissues.

Mori et al suggested that an autocrine stimulatory loop involving major basic protein, a product of eosinophil degranulation, IL-6, which enhances collagen production and is induced by major basic protein, and TGF-beta could account for the progressive fibrosis seen in several eosinophil-prominent disorders. [9]

Other studies showed elevated levels of serum manganese superoxide dismutase and tissue metalloproteinase 1 (TIMP-1) in eosinophilic fasciitis, suggesting a role in pathogenesis and providing a possible marker of disease activity. [10]

Fasciitis may be a common manifestation of various pathophysiologic processes associated with eosinophilia. 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.

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Etiology

The etiology of eosinophilic fasciitis is unknown. Its clinical manifestations are the result of an inflammatory response in the affected tissues. As noted above, the current understanding of eosinophilic fasciitis relies on a relatively few case series and case reports. As such, many etiologic factors have been suggested with varying degrees of supporting evidence. Any of these factors, alone or in combination, may possibly initiate this inflammatory response.

Several possible triggers have been reported with some consistency. A preceding history of vigorous exercise or trauma has been reported in 30%-50% of patients. [11, 12] Multiple drugs have also been implicated, including simvastatin, atorvastatin, and phenytoin. [13, 14, 15] In addition, cases of eosinophilic fascitis following treatment with immunne checkpoint inhibitors atezolizumab, nivolumab and pembrolizumab have been reported. [16, 17] One report described eosinophilic fasciitis in association with cemiplimab. [18]

In several cases, patients have demonstrated positive Borrelia serologies. The significance of this finding continues to be debated. Spirochetes were visualized by silver stain in 4 patients in one study. [19] These findings have not been repeated. It has been suggested that positive serology for Borrelia represents an epiphenomenon among cases from _Borrelia_-endemic areas and is insufficient evidence of infection and therefore does not support a causal association. [20]

Eosinophilic fasciitis shares clinical similarities, as well as key differences, with eosinophilia-myalgia syndrome. Some studies have suggested an association between L-tryptophan ingestion and eosinophilic fasciitis. [21, 22] Despite this, no consistent association has been found between L-tryptophan or other dietary exposure and eosinophilic fasciitis. As evidence, l-tryptophan use was significantly associated with dyspnea, an uncommon finding in eosinophilic fasciitis cases. In another instance, a patient with eosinophilic fasciitis had used L-tryptophan for several years but had started a formal exercise program 2 weeks prior to disease onset. [23]

Multiple additional etiologic triggers have been suggested by single or infrequent case reports.

Eosinophilic fasciitis has been associated with several diseases. [24] Hematologic diseases have been consistently reported and are supported by large case series and case reports. [11, 12, 25] The spectrum of associated hematologic disease is broad and includes aplastic and hemolytic anemia, thrombocytopenia, myeloproliferative disorders, myelodysplastic disorders, lymphoma, leukemia, monoclonal gammopathy of undetermined significance (MGUS), and multiple myeloma. [25, 26, 27]

An association with thyroid disease has been reported in several cases. [28] Eosinophilic fasciitis has rarely been linked to solid-organ tumors and primary biliary cholangitis, in addition to several other diseases. These disease associations may suggest a shared pathophysiology of cellular dysregulation and/or autoimmunity.

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Epidemiology

Frequency

Eosinophilic fasciitis is very rare.

Mortality/Morbidity

No data are available on morbidity or mortality rates associated with eosinophilic fasciitis. Morbidity may result from joint contractures or carpal tunnel syndrome associated with fascial fibrosis. Rarely, a fatal aplastic anemia may develop.

Race-, sex-, and age-related demographics

Eosinophilic fasciitis affects Whites more often than it affects other races. It has been reported in African Americans, Africans, and Asians. [29, 30, 31, 32, 33]

In adults, eosinophilic fasciitis affects women more often than men. [11, 34, 12]

The age range in eosinophilic fasciitis is 1-88 years, although most patients present during the third to sixth decades of life. [34] The average age of onset in two case series was 54.4 and 49.8 years. [34, 12]

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Prognosis

A retrospective review found that clinical factors associated with persistent fibrosis included the following [35] :

Loss of edema is usually the first clinical sign of improvement and can occur within 4 weeks of commencing treatment. Concurrently, the skin becomes softer, but 3-6 months may elapse before maximal reduction in induration and contractures is achieved. [11, 12]

While total resolution of the clinical signs can occur, some degree of induration remaining even after many months of corticosteroid therapy is not unusual.

A direct correlation does not always exist between clinical disease activity and laboratory findings. The eosinophil count and erythrocyte sedimentation rate (ESR) usually return to reference ranges within 6-8 weeks, although ESR prolongation and hypergammaglobulinemia may persist for up to 12 months. [11, 12]

Eventually, corticosteroid therapy can be withdrawn in many patients, without relapse occurring.

The development of aplastic anemia is a rare but grave complication. [36] One study reported on 4 patients with eosinophilic fasciitis and severe aplastic anemia. In 3 cases, the aplastic anemia was refractory to conventional immunosuppressive therapy with antithymocyte globulin and cyclosporine. However, in 1 patient, rituximab had significant efficacy for both the skin and hematologic symptoms. In an additional 19 cases of eosinophilic fasciitis and aplastic anemia, corticosteroid regimens improved skin symptoms in 5 of 12 cases but were ineffective in the treatment of aplastic anemia in all but 1 case. Aplastic anemia was profound in 13 cases and was the cause of death in 8 cases. Only 5 patients achieved long-term remission. [37]

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Author

Peter M Henning, DO MAJ, US Marine Corps; Rheumatology Clinic, Madigan Army Medical Center

Peter M Henning, DO is a member of the following medical societies: American College of Physicians

Disclosure: Nothing to disclose.

Coauthor(s)

George R Mount, MD MAJ USA MC, Assistant Professor of Medicine, Uniformed Services University of the Health Sciences; Attending Physician, Department of Rheumatology, Madigan Army Medical Center, Tacoma, WA

George R Mount, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, Phi Beta Kappa

Disclosure: Nothing to disclose.

Kristine M Lohr, MD, MS Professor, Department of Internal Medicine, Interim Chief, Division of Rheumatology, Director, Rheumatology Training Program, University of Kentucky College of Medicine

Kristine M Lohr, MD, MS is a member of the following medical societies: American College of Physicians, American College of Rheumatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Lawrence H Brent, MD Associate Professor of Medicine, Sidney Kimmel Medical College of Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center

Lawrence H Brent, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Physicians, American College of Rheumatology

Disclosure: Stock ownership for: Johnson & Johnson.

Additional Contributors

Acknowledgements

The opinions or assertions contained here are the private views of the authors and are not to be construed as official or as reflecting the views of the Department of the Army or the Department of Defense.