Surgical Intervention for anti-laminin 332 mucous membrane pemphigoid with larynx involvement

A 44-years old male patient presented in spring 2017 with disseminated blisters and erosions at the nasal, oral and ocular mucosa. Based on the histological, direct immunofluorescent and serological findings an anti-laminin 332 mucous membrane pemphigoid (MMP) was diagnosed. An extended tumor examination led to non-pathological findings.

Although the Azathioprine therapy improved the skin condition, the mucosal lesions required a continuous high-dose prednisolone therapy, 6 cycles of immunoadsorption, an oral therapy with Dapsone, intravenous immunoglobulin and mycophenolate mofetil (MMF). Yet, the patient presented an increasing dyspnea, cough, hoarseness and stridor due to a pronounced stenosis in the glottis area. A surgical approach was discountenanced by most of the ENT departments the patient visited, due to the fear of relapsing scar tissue.

After carefully discussing different therapeutic options with the patient, a microlaryngoscopy was conducted which showed a swelling of the interarytenoid region with a remaining glottic opening of only 1 – 2 mm, leading to the progressive dyspnea and inspiratory Stridor. The symptoms subsided completely after glottis-expanding transection of the stenosis using a CO2 laser in superpulse mode under flow-apnea ventilation. To prevent renewed scarring, lateral fixation (Lichtenberg's technique) of the right vocal cord followed. Postoperatively and by continuing the therapy of prednisolone and MMF the patient reached a complete clinical and serological remission.

Laryngeal involvement is described in 12% of MMP. Those cases require not only conservative therapy to eliminate autoantibodies but also surgical intervention to treat scar related complications. A lateral fixation may effectively resolve the problem of recurring scar tissue.

Publication History

Publication Date:
23 April 2019 (online)