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

Updated: Dec 15, 2017
  • Author: Claudia Hernandez, MD, FAAD; Chief Editor: William D James, MD  more...
  • Print
Presentation

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
Next:

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 Magnified view of papules and nodules with central necrosis.
Distal digital purpuric papule. Distal digital purpuric papule.
Previous
Next:

Complications

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

Previous