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Dermatologic Manifestations of Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome)

  • Author: Claudia Hernandez, MD, FAAD; Chief Editor: William D James, MD  more...
 
Updated: Oct 16, 2015
 

Background

Eosinophilic granulomatosis with polyangiitis (EGPA) (alternatively termed Churg-Strauss syndrome or allergic granulomatosis and angiitis) is a rare disorder characterized by a small- and medium-sized vessel vasculitis with severe asthma and tissue eosinophilia.[1] The combination of allergic granulomatosis and angiitis associated with asthma, typically of adult onset, and allergic rhinitis[2] was first described by Churg and Strauss in 1951, when they reviewed 13 autopsy cases that were previously classified as polyarteritis nodosa.[3] These cases were atypical in that asthma and eosinophilia preceded the systemic vasculitis. They named the syndrome "allergic angiitis and allergic granulomatosis," which came to be known as Churg-Strauss syndrome (CSS) and is now EGPA.[3] Since the identification of antineutrophil cytoplasmic antibodies (ANCA) in the early nineties, EGPA is part of a group of diseases known as the ANCA-associated vasculitides (AAV) that includes granulomatosis with polyangiitis (previously known as Wegener granulomatosis) and microscopic polyangiitis.[4]

Also see Churg-Strauss Syndrome (rheumatology focus) and Churg-Strauss Disease (neurology focus).

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Pathophysiology

The diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA) is challenging because of the highly variable presentation and course of the disease. Some patients have only mild manifestations, while others are affected by life-threatening conditions. Some investigators have divided EGPA into 3 phases, as follows[5] :

  1. A prodromal phase characterized by allergic manifestations followed by asthma
  2. A second phase of marked peripheral blood eosinophilia and eosinophilic tissue infiltration that produces a picture similar to that of Loeffler syndrome, chronic eosinophilic pneumonia, or eosinophilic gastroenteritis
  3. A third phase with systemic vasculitis

Pulmonary involvement, neuropathy, and skin lesions are common with each occurring in at least two thirds or more of affected patients. Other systemic features include polyneuropathy (symmetric or mononeuritis multiplex), ischemic bowel disease, nasal perforation, glomerulonephritis, ocular inflammation, coronary arteritis, and cardiomyopathy.[6] Myocardial involvement or congestive heart failure is the most common cause of death. A high eosinophilia count is present in all patients, averaging 1 X 109/L, and approximately two thirds have a positive perinuclear ANCA titer, which targets primarily myeloperoxidase.[7]

More than one classification scheme exists for EGPA, including Lanham’s criteria, which emphasize clinical features, and the Chapel Hill Consensus Conference criteria, which emphasize pathology. A third option is the American College of Rheumatology (ACR) criteria, originally created for epidemiologic and therapeutic studies.

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Epidemiology

United States

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare and likely underreported disease.

International

EGPA is a rare disease. The annual incidence of the disorder is estimated at 2.4-4 cases per million general population, while among asthma patients, an average of 34.6 cases per million asthma population as been reported.[8]

Race

No racial predilection is currently recognized for EGPA.

Sex

Sexual predilection for EGPA varies according to the source, with most sources citing a male predominance. The male-to-female ratio is 1.3:1.

Age

EGPA may affect both children and elderly persons. The age of onset is wide (4-75 y), with a mean age of 38 years.[9]

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Contributor Information and Disclosures
Author

Claudia Hernandez, MD, FAAD Associate Professor, Director of Dermatological and Clinical Studies, Department of Dermatology, University of Illinois at Chicago College of Medicine; Attending Physician, University of Illinois at Chicago Hospital

Claudia Hernandez, MD, FAAD is a member of the following medical societies: American Academy of Dermatology, American Association for Cancer Research, Association of Professors of Dermatology, Chicago Dermatological Society, Chicago Medical Society, Illinois Dermatological Society, Society for Pediatric Dermatology, Women's Dermatologic Society

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

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

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

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

Disclosure: Nothing to disclose.

Additional Contributors

Jacek C Szepietowski, MD, PhD Professor, Vice-Head, Department of Dermatology, Venereology and Allergology, Wroclaw Medical University; Director of the Institute of Immunology and Experimental Therapy, Polish Academy of Sciences, Poland

Disclosure: Received consulting fee from Orfagen for consulting; Received consulting fee from Maruho for consulting; Received consulting fee from Astellas for consulting; Received consulting fee from Abbott for consulting; Received consulting fee from Leo Pharma for consulting; Received consulting fee from Biogenoma for consulting; Received honoraria from Janssen for speaking and teaching; Received honoraria from Medac for speaking and teaching; Received consulting fee from Dignity Sciences for consulting; .

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous authors, Paula Vogel, MD, and Daniel Schissel, MD, to the development and writing of this article.

References
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  6. Gota CE, Mandell BF. Systemic necrotizing vasculitis. Wolff K, Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ. Fitzpatrick's Dermatology in General Medicine. 7th. New York: McGraw Hill; 2008. 2: 1606-1616/165.

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  9. Lhote F, Guillevin L. Polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome. Clinical aspects and treatment. Rheum Dis Clin North Am. 1995 Nov. 21(4):911-47. [Medline].

  10. Neumann T, Manger B, Schmid M, Kroegel C, Hansch A, Kaiser WA. Cardiac involvement in Churg-Strauss syndrome: impact of endomyocarditis. Medicine (Baltimore). 2009 Jul. 88(4):236-43. [Medline].

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  19. Kaushik VV, Reddy HV, Bucknall RC. Successful use of rituximab in a patient with recalcitrant Churg-Strauss Syndrome. Ann Rheum Dis. 2006 Aug. 65(8):1116-7. [Medline].

  20. Josselin-Mahr L, Werbrouck-Chiraux A, Garderet L, Cabane J. Efficacy of imatinib mesylate in a case of Churg-Strauss syndrome: evidence for the pathogenic role of a tyrosine kinase?. Rheumatology (Oxford). 2013 Jul 27. [Medline].

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Granuloma with a central core of eosinophilic debris surrounded by a peripheral palisade of epithelioid histiocytes and eosinophils.
Bilateral papules and nodules with central necrosis.
Magnified view of papules and nodules with central necrosis.
Distal digital purpuric papule.
 
 
 
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