Dermatologic Manifestations of Eosinophilic Fasciitis Clinical Presentation

  • Author: Brad S Graham, MD; Chief Editor: William D James, MD   more...
 
Updated: Aug 16, 2011
 

History

  • Eosinophilic fasciitis patients present with the sudden onset of painful, tender, edematous, and erythematous extremities.
    • The disorder progresses rapidly; within weeks to months, patients develop stiffness and sclerodermatous induration, resulting in characteristic flexion contractures and impaired mobility.
    • The forearms, the upper arms, the lower legs, the thighs, and the trunk are involved (in order of decreasing frequency).
  • As many as 50% of patients report an episode of strenuous physical exercise or activity immediately preceding the onset of the illness.
  • Malaise, weakness, and fever are frequently present. A study in 2008 addressing the physical burden noted that fatigue was the most common symptom. Pain and itch were also frequent complaints. Of all patients, 62% noted fatigue, pain, and itch.[9]
  • Overt arthritis occurs in as many as 40% of patients.
  • Symptoms of carpal tunnel syndrome have been reported.
  • Visceral involvement and Raynaud phenomenon are rare.
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Physical

The clinical presentation of eosinophilic fasciitis evolves through 3 stages; the various stages present simultaneously in different areas of the body. The first stage presents with symmetric, diffuse, erythematous tenderness of the extremities, followed by an edematous phase that produces a coarsely dimpled appearance (cobblestoning) or a finely dimpled appearance (peau d'orange). The last phase involves rippling of the skin with areas of hypopigmentation, induration, and skin tightness.

Lower back part of the legs shows hypopigmentationLower back part of the legs shows hypopigmentation, induration, biopsy site, and asymmetric involvement. Posterior thigh shows woody induration, sclerosis,Posterior thigh shows woody induration, sclerosis, and hypopigmentation. Close-up view of left posterior thigh 2 weeks lateClose-up view of left posterior thigh 2 weeks later shows erythema, scaling, alopecia, and rippled induration. Posterior part of the calf in the first week of ilPosterior part of the calf in the first week of illness shows erythema, edema, alopecia, scaling, and early induration. The right calf is relatively uninvolved with patchy erythema only. Photograph of the posterior part of the calf at 3 Photograph of the posterior part of the calf at 3 weeks shows complete sclerosis and induration with patchy erythema.

In severely affected areas, both the skin and the subcutaneous tissues are bound-down and inseparable from the underlying muscle, and they have a woody-type appearance. With elevation of the involved extremities, furrows along the course of the superficial veins may be present; this finding is referred to as the groove sign. Although the extremities are preferentially involved (88%), the trunk may be involved. The hands, the feet, and the face are spared.

  • Joint contractures of the elbows, wrists, ankles, knees, and shoulders may be found in 55-75% of patients. Unilateral involvement has been reported.[10]
  • Carpal tunnel syndrome is present in 20% of patients.
  • Inflammatory arthritis is present in as many as 40% of patients.
  • Subclinical myositis is present in a minority of patients.
  • A concurrent localized lesion of morphea may be seen in 25% of patients.
  • In contrast to scleroderma, Raynaud phenomenon, abnormal nail fold capillaries, and sclerodactyly are not present. Visceral involvement is rare, with few reports of involvement of the lungs, the esophagus, and the myocardium.
  • Primary presentation as angioedema was also reported in a patient.[11]
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Causes

See Pathophysiology.

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Contributor Information and Disclosures
Author

Brad S Graham, MD  Consulting Staff, Dermatology Associates of Tyler

Brad S Graham, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society of Dermatopathology, and Texas Dermatological Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Ponciano D Cruz Jr, MD  Vice-Chair, JB Shelmire Professor, Department of Dermatology, University of Texas Southwestern Medical Center

Ponciano D Cruz Jr, MD is a member of the following medical societies: Texas Medical Association

Disclosure: RCTS Consulting fee Independent contractor; Mary Kay Cosmetics Honoraria Consulting; Galderma Grant/research funds Principal Investigator

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Edward F Chan, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine

Edward F Chan, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD  Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System

William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology

Disclosure: Elsevier Royalty Other

References

  1. DeGiovanni C, Chard M, Woollons A. Eosinophilic fasciitis secondary to treatment with atorvastatin. Clin Exp Dermatol. Jan 2006;31(1):131-2. [Medline].

  2. Serrano-Grau P, Mascaró-Galy JM, Iranzo P. Eosinophilic fasciitis after taking simvastatin. Actas Dermosifiliogr. Jun 2008;99(5):420-1. [Full Text].

  3. Anton E. Failure to demonstrate Borrelia burgdorferi-specific DNA in lesions of eosinophilic fasciitis. Histopathology. Jul 2006;49(1):88-90. [Medline].

  4. Jinnin M, Ihn H, Yazawa N, Asano Y, Yamane K, Tamaki K. Elevated serum levels of manganese superoxide dismutase in patients with eosinophilic fasciitis. Clin Rheumatol. Dec 2003;22(6):505. [Medline].

  5. Jinnin M, Ihn H, Yamane K, Asano Y, Yazawa N, Tamaki K. Serum levels of tissue inhibitor of metalloproteinase-1 and 2 in patients with eosinophilic fasciitis. Br J Dermatol. Aug 2004;151(2):407-12. [Medline].

  6. Sherber NS, Wigley FM, Paget SA. Diffuse fasciitis with eosinophilia developing after local irradiation for breast cancer. Clin Rheumatol. Jun 2009;28(6):729-32. [Medline].

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  8. Endo Y, Tamura A, Matsushima Y, Iwasaki T, Hasegawa M, Nagai Y. Eosinophilic fasciitis: report of two cases and a systematic review of the literature dealing with clinical variables that predict outcome. Clin Rheumatol. Sep 2007;26(9):1445-51. [Medline].

  9. Kroft EB, de Jong EM, Evers AW. Physical burden of symptoms in patients with localized scleroderma and eosinophilic fasciitis. Arch Dermatol. Oct 2008;144(10):1394-5.

  10. Daniel RS, Brown AN. Case report of unilateral eosinophilic fasciitis in a Vietnamese woman. Am J Med Sci. Feb 2009;337(2):153-4. [Medline].

  11. Dowden AM, Ballas ZK. Eosinophilic fasciitis masquerading as angioedema. Ann Allergy Asthma Immunol. Mar 2009;102(3):258-9. [Medline].

  12. Varga J, Griffin R, Newman JH, Jimenez SA. Eosinophilic fasciitis is clinically distinguishable from the eosinophilia-myalgia syndrome and is not associated with L-tryptophan use. J Rheumatol. Feb 1991;18(2):259-63. [Medline].

  13. Varga J, Kahari VM. Eosinophilia-myalgia syndrome, eosinophilic fasciitis, and related fibrosing disorders. Curr Opin Rheumatol. Nov 1997;9(6):562-70. [Medline].

  14. Baumann F, Bruhlmann P, Andreisek G, Michel BA, Marincek B, Weishaupt D. MRI for diagnosis and monitoring of patients with eosinophilic fasciitis. AJR Am J Roentgenol. Jan 2005;184(1):169-74. [Medline].

  15. Valencia IC, Chang A, Kirsner RS, Kerdel FA. Eosinophilic fasciitis responsive to treatment with pulsed steroids and cyclosporine. Int J Dermatol. May 1999;38(5):369-72. [Medline].

  16. Romano C, Rubegni P, De Aloe G, et al. Extracorporeal photochemotherapy in the treatment of eosinophilic fasciitis. J Eur Acad Dermatol Venereol. Jan 2003;17(1):10-3. [Medline].

  17. Tzaribachev N, Holzer U, Schedel J, Maier V, Klein R, Kuemmerle-Deschner J. Infliximab effective in steroid-dependent juvenile eosinophilic fasciitis. Rheumatology (Oxford). Jun 2008;47(6):930-2. [Medline].

  18. Haiduc VF, Erkan D, Kirou K, Birchansky S, Park J, Danon MJ. Anti-neutrophil cytoplasmic antibody (c-ANCA) positive recurrent eosinophilic fasciitis responsive to cyclophosphamide: a clinical pathology conference held by the Division of Rheumatology at Hospital for Special Surgery. HSS J. Feb 2008;4(1):81-6. [Medline]. [Full Text].

  19. Smith LC, Cox NH. Dapsone treatment for eosinophilic fasciitis. Arch Dermatol. Jul 2008;144(7):845-7. [Medline].

  20. Weber HO, Schaller M, Metzler G, Röcken M, Berneburg M. Eosinophilic fasciitis and combined UVA1--retinoid--corticosteroid treatment: two case reports. Acta Derm Venereol. 2008;88(3):304-6. [Medline].

  21. Horacek E, Sator PG, Gschnait F. 'Venous furrowing': a clue to the diagnosis of eosinophilic fasciitis. A case of eosinophilic fasciitis ultimately treated with oral PUVA therapy. Dermatology. 2007;215(1):89-90. [Medline].

  22. Khanna D, Agrawal H, Clements PJ. Infliximab may be effective in the treatment of steroid-resistant eosinophilic fasciitis: report of three cases. Rheumatology (Oxford). Jun 2010;49(6):1184-8. [Medline]. [Full Text].

  23. Pimenta S, Bernardes M, Bernardo A, Brito I, Castro L, Simões-Ventura F. Intravenous immune globulins to treat eosinophilic fasciitis: a case report. Joint Bone Spine. Oct 2009;76(5):572-4. [Medline].

  24. Kitamura Y, Hatamochi A, Hamasaki Y, Ikeda H, Yamazaki S. Association between eosinophilic fasciitis and systemic lupus erythematosus. J Dermatol. Feb 2007;34(2):150-2. [Medline].

  25. Khalifa M, Slim I, Ghannouchi N, et al. Eosinophilic fasciitis and toxic thyroid adenoma--a novel association. Acta Clin Belg. May-Jun 2008;63(3):197-9.

  26. Tahara K, Yukawa S, Shoji A, Hayashi H, Tsuboi N. Long-term remission by cyclosporine in a patient with eosinophilic fasciitis associated with primary biliary cirrhosis. Clin Rheumatol. Sep 2008;27(9):1199-201. [Medline].

 
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Lower back part of the legs shows hypopigmentation, induration, biopsy site, and asymmetric involvement.
Posterior thigh shows woody induration, sclerosis, and hypopigmentation.
Close-up view of left posterior thigh 2 weeks later shows erythema, scaling, alopecia, and rippled induration.
Posterior part of the calf in the first week of illness shows erythema, edema, alopecia, scaling, and early induration. The right calf is relatively uninvolved with patchy erythema only.
Photograph of the posterior part of the calf at 3 weeks shows complete sclerosis and induration with patchy erythema.
Note the marked thickening and replacement of the entire dermis with sclerotic collagen on this incisional biopsy sample from the left posterior part of the thigh.
Photomicrograph of subcutaneous fat-fascia junction shows entrapment of subcutaneous fat by intersecting thick bands of fibrosis. Thickening and fibrosis of fascia and lymphoid aggregates are seen.
Photomicrograph of fascia-skeletal muscle junction shows markedly thickened fascia with heavy inflammatory infiltration.
High-power photomicrograph of fascia shows heavy inflammatory infiltration with numerous eosinophils, lymphocytes, and occasional plasma cells.
 
 
 
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