History

The 3 phases—allergic, eosinophilic, and vasculitic—of eosinophilic granulomatosis with polyangiitis (EGPA) do not necessarily follow one another in any particular order and frequently overlap. Symptoms depend on the phase and organ systems involved. A careful history should include medication usage, infectious symptoms, and/or preexisting disease.^{[15, 11]}

The allergic phase (also called prodromal) may last months to years and is characterized by the following:

Rhinitis, sinus pain, headache
Cough
Wheezing or asthma is one of the main manifestations, with an estimated 96-100% of patients affected
Arthralgias, myalgias
Malaise
Fever
Weight loss

The eosinophilic phase (or second phase) is characterized by the following:

Peripheral eosinophilia (usually greater than 1,500/µL) and possible organ involvement
Gastrointestinal - Abdominal pain, diarrhea, bleeding, nausea, vomiting, bleeding
Pulmonary - Cough

The vasculitic phase (or third phase) is characterized by the following:

General - Malaise, fever
Cardiac - Chest pain, dyspnea
Cutaneous - Purpura, papules
Pulmonary - Cough, hemoptysis
Rheumatologic - Arthralgia, arthritis, myalgia
Neurologic - Weakness, numbness

Physical Examination

Clinical findings in eosinophilic granulomatosis with polyangiitis (EGPA) vary depending on the phase and organ systems involved.^{[2, 11, 16]}

Allergic (prodromal) phase findings are as follows:

Upper respiratory tract symptoms affect majority of patients
Nasal polyps
Wheezing
Cough
Rhinitis
Recurrent or chronic sinusitis

Eosinophilic (second) phase findings are as follows:

General - Weight loss, fever, sweats
Pulmonary - Cough, hemoptysis, rales, rhonchi
Gastrointestinal - Rebound, masses, obstruction, ascites, bleeding

Vasculitic (third) phase findings are as follows:

Most commonly affects skin and peripheral nervous system ^[17]
Constitutional symptoms - Fever, weight loss, adenopathy
Cardiac - Gallop, pericardial friction rub, jugular venous distension, peripheral edema
Pulmonary - Rales, rhonchi
Nervous system - Mononeuritis multiplex affects up to 75% of patients (wrist or foot drop typical), ^[17]mixed sensorimotor peripheral neuropathy (most often in a glove-and-stocking distribution), ^[18]loss of a visual field, cerebral hemorrhage, ^[19]infarction
Renal - Mild proteinuria and hematuria (more common in those with serologically detectable ANCA)
Genitourinary - Obstructive uropathy
Ocular - Episcleritis, panuveitis, marginal corneal ulceration, conjunctival infiltration, retinal infarction
Musculoskeletal - Joint swelling, muscle tenderness
Cutaneous - Palpable purpura (50%), subcutaneous nodules (scalp and extensor surfaces on extremities), firm papules on fingertips (may resemble septic emboli), urticaria, livedo reticularis

See the images below.

Magnified view of papules and nodules with central necrosis.

View Media Gallery

Distal digital purpuric papule.

View Media Gallery

Complications

Although many organ systems can be affected by eosinophilic granulomatosis with polyangiitis (EGPA), patients are most likely to have pulmonary or cardiac disease.

Differential Diagnoses

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Media Gallery

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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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 Former 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: Alpha Omega Alpha, American Academy of Dermatology, American Society for MOHS Surgery, Association of Military Dermatologists, 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: Received income in an amount equal to or greater than $250 from: Elsevier; WebMD<br/>Served as a speaker for various universities, dermatology societies, and dermatology departments.

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.