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Updated: Aug 4, 2026
10:27
Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis
Published on: December 15, 2011
[Unusual presentation forms of Wegener's disease]
M T Lahoz Zamarro1, J Martínez Subías, G Martínez Sanz
1Servicio de ORL, Hospital Obispo Polanco de Teruel, Argentina.
Acta Otorrinolaringologica Espanola
|June 14, 2003
Summary
Wegener's granulomatosis, a form of granulomatous necrotizing vasculitis, can manifest in localized or generalized ways. This study reports three atypical cases, highlighting varied presentations and long-term disease control.
Area of Science:
- Medicine
- Rheumatology
- Pathology
Background:
- Wegener's granulomatosis is characterized by granulomatous necrotizing vasculitis.
- It typically affects the respiratory tract and kidneys.
- The disease can present in generalized or localized forms.
Observation:
- Three atypical cases of Wegener's granulomatosis were observed.
- Case 1: Limited form with isolated airway involvement.
- Case 2: Otitis with cranial nerve (I, II, V, VI, VII, VIII) progression.
- Case 3: Generalized form with sinusitis and orbital pseudotumor.
Findings:
- Atypical presentations of Wegener's granulomatosis can occur.
- Localized forms may remain confined to the initial site.
- Long-term follow-up (22 and 14 years) showed sustained control in two cases without further disease progression.
Implications:
- Recognizing atypical presentations is crucial for timely diagnosis.
- Localized Wegener's granulomatosis may have a favorable long-term prognosis.
- Understanding diverse manifestations aids in managing this rare vasculitis.
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