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

Updated: Aug 09, 2022
  • Author: Claudia Hernandez, MD, FAAD; Chief Editor: William D James, MD  more...
  • Print

Physical Examination

Clinical findings in eosinophilic granulomatosis with polyangiitis (EGPA) vary depending on the phase and organ systems involved. [2, 6, 18]

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 [19]
  • 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), [19] mixed sensorimotor peripheral neuropathy (most often in a glove-and-stocking distribution), [20] loss of a visual field, cerebral hemorrhage, [21] 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.

A representative case of EGPA was reported by Venade et al. A 37-year-old man presented with fever, productive cough, occasional dyspnea, anorexia, and lethargy. The patient was discovered to have leukocytosis and eosinophilia, and he was also determined to be in acute renal failure. Treatment was 3 boluses of methylprednisolone 1 g and commencement of cyclophosphamide therapy with resolution of symptoms and restoration of renal function. [22]