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A Rare Case of Granulomatosis With Polyangiitis With Cutaneous Pyoderma Gangrenosum-Like Lesions of the Bilateral

Spencer H Short1, Michael McGlone1, Jason F Ohlstein1

  • 1Department of Otolaryngology - Head & Neck Surgery, Lehigh Valley Health Network, Allentown, PA, USA.

Ear, Nose, & Throat Journal
|July 14, 2025
PubMed

Insights

Granulomatosis with polyangiitis (GPA) can present with rare skin lesions affecting the parotid glands. Early diagnosis and treatment with Rituximab led to symptom resolution in this case.

Area of Science:

  • Rheumatology
  • Dermatology
  • Otolaryngology

Background:

  • Granulomatosis with polyangiitis (GPA), previously Wegener's granulomatosis, is a rare small vessel vasculitis.
  • GPA typically involves necrotizing granulomas in multiple organs, but presentations can vary widely.

Purpose of the Study:

  • To report a rare case of GPA presenting with pyoderma gangrenosum-like lesions on the parotid glands.
  • To highlight the diagnostic challenges and treatment outcomes in such atypical GPA presentations.

Main Methods:

  • Case report of a 79-year-old male with progressive ulcerative lesions.
  • Diagnostic workup included punch biopsy, imaging (chest radiograph), and autoimmune serology.
  • Treatment involved Rituximab therapy.

Main Results:

  • The patient presented with painful, necrotic parotid gland lesions progressing to salivary fistulas and facial nerve weakness.
  • Biopsy revealed granulomatous inflammation; lab work showed elevated autoimmune markers including ANCA.
  • Treatment with Rituximab resulted in resolution of cutaneous ulcerations and facial nerve palsy.

Conclusions:

  • This case illustrates a rare manifestation of GPA involving the parotid glands and facial nerve.
  • GPA should be considered in the differential diagnosis of necrotic skin lesions, even with unusual locations.
  • Timely diagnosis and appropriate immunosuppressive therapy are crucial for managing GPA complications.
Keywords:
facial nervehead and neckotolaryngologyparotid

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