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

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

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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