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Wegener's granulomatosis presenting as pyoderma gangrenosum
Hajnal Irén Szõcs1, Katalin Torma, Edina Petrovicz
1Department of Dermato-Venereology, Semmelweis University, Budapest, Hungary.
International Journal of Dermatology
|November 26, 2003
Summary
Wegener's granulomatosis can present with skin ulcers and affect the lungs and orbits. Prompt treatment with methylprednisone and cyclophosphamide resolved severe symptoms in this case.
Area of Science:
- Rheumatology
- Dermatology
- Otorhinolaryngology
Background:
- Pyoderma gangrenosum is a rare, ulcerative neutrophilic dermatosis.
- Wegener's granulomatosis (now Granulomatosis with Polyangiitis) is a systemic vasculitis affecting small to medium-sized vessels.
Observation:
- A 59-year-old male presented with a necrotizing shin ulcer, initially diagnosed as pyoderma gangrenosum.
- Symptoms progressed to include nasal discharge, fever, cough, lung inflammation, orbital granulomas, and positive c-ANCA.
- Clinical presentation suggested a rare overlap or progression to Wegener's granulomatosis.
Findings:
- The patient exhibited characteristic signs of Wegener's granulomatosis, including upper respiratory tract, lung, and orbital involvement.
- Diagnostic findings included positive c-ANCA, elevated serum IgA, microhaematuria, and proteinuria.
- Histopathology confirmed granulomatous inflammation.
Implications:
- This case highlights the importance of considering systemic vasculitis in atypical pyoderma gangrenosum presentations.
- Early diagnosis and aggressive immunosuppressive therapy (methylprednisone, cyclophosphamide) are crucial for managing severe Wegener's granulomatosis.
- Successful treatment led to the resolution of both cutaneous and systemic manifestations.