Extensive supraglottic swelling

Introduction:

Melkersson-Rosenthal syndrome (MRS) is a neuromucocutaneous granulomatous disease characterized by recurrent swelling of the lips, especially the lower lip (Cheilitis granulomatosa Miescher), intermittend facial palsy and lingua plicata in 20% of the cases. Laryngeal manifestations are not described so far.

Methods:

Case report. A 61-year-old female patient complained of stridor and dysphonia, progressive during the last months. MRS was already known in her history. The patient also reported a history of tuberculosis in adolescence.

Results:

The patient needed to be hospitalized due to an extensive supraglottic laryngeal edema. Initially a conservative therapeutic approach with intravenous antibiotics, high-dose prednisolone and suprarenin inhalation was applied. Since this did not lead to improvement, an endoscopic laser-assisted surgical debulking of swollen mucosa in the aryepiglottic areas was performed. Smears were taken intraoperatively and the material was collected for histopathological and microbiological examination, also for the definitive exclusion of tuberculosis. Histologically, a dominant lymphocytic chronic inflammation with consecutive stromal edema and lymphangiectasis was described, there were no hints for sarcoidosis nor tuberculosis.

Conclusions:

Despite missing epitheloid cell granulomas, the histopathologic findings were described compatible with MRS. Especially in early stages typical granulomas can rarely be detected. Clear clinical symptoms with unexpected swelling as well as treatment-failures or a relapsing course of the disease underline MRS and are in agreement with the case described here.

Publication History

Publication Date:
23 May 2018 (online)