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[Buccal lymphomatoid granulomatosis].
J Cabane1, P Godeau, G Chomette
1Service de Médecine Interne, hôpital de la Pitié, Paris.
La Revue De Medecine Interne
|January 1, 1990
Summary
Lymphomatoid granulomatosis, a rare condition, can be diagnosed from mouth ulcers even without lung nodules. Effective treatment with cyclophosphamide and prednisone led to long-term remission in this case.
Area of Science:
- Immunopathology
- Oncology
- Granulomatous Diseases
Background:
- Lymphomatoid granulomatosis is a rare systemic vasculitis characterized by granulomatous inflammation and atypical lymphoid cells.
- Diagnosis can be challenging, often requiring a combination of clinical, histological, and immunophenotypic findings.
- Classical presentation involves pulmonary nodules, but extrathoracic manifestations can occur.
Observation:
- A 57-year-old man presented with refractory, multifocal oral ulcers.
- Biopsies revealed a polymorphous granuloma with atypical lymphoid cells (OKT3+, OKT4+) and an angiocentric pattern.
- The patient also exhibited cardiac rhythm disturbances and proteinuria, prompting suspicion of lymphomatoid granulomatosis despite absent pulmonary nodules.
Findings:
- The patient responded well to cyclophosphamide and prednisone, with a minor relapse controlled by adjusting cyclophosphamide dosage.
- Complete remission was achieved and sustained for over four years after treatment cessation.
- Histopathological findings from oral ulcers were sufficient for diagnosis and guiding treatment.
Implications:
- This case demonstrates that lymphomatoid granulomatosis can be diagnosed via oral mucosal biopsy, even in the absence of pulmonary involvement.
- Early diagnosis and treatment of extrathoracic lymphomatoid granulomatosis are crucial for favorable outcomes.
- The successful response to immunosuppressive therapy suggests a potentially curable granulomatous process rather than a true lymphoma.