General anesthetic management for diagnostic splenectomy in a patient with paraneoplastic pemphigus

Paraneoplastic pemphigus (PNP) is an autoantibody-mediated mucocutaneous blistering disease that often occurs with confirmed or occult malignancy. It is often accompanied by extensive eruption of respiratory mucosa and can lead to respiratory failure. The indications for anesthetic management in patients with PNP are rare due to the high risk of generating further eruption of respiratory mucosa during intubation, with potentially disastrous consequences. We report the anesthetic management in a 43-year-old woman with PNP scheduled for diagnostic splenectomy. The surgery was postponed due to persistent hoarseness, diffuse oral-pharyngeal mucositis, and laryngeal erosion. Methylprednisolone 40 mg 3 times daily was prescribed for controlling the mucosa lesions. Two weeks later, after the improvement of mucosal lesions, direct laryngoscopic intubation and the scheduled splenectomy were performed under general anesthesia. Pathology of the spleen showed low-grade B-cell lymphoma had induced PNP. Flexible fiberoptic bronchoscopic examination revealed neither bleeding nor bullae formation in the trachea either immediately after intubation or before extubation. The tracheal mucosa is highly susceptible to mechanical stimulation with direct laryngoscopic intubation. Cuff inflation and positive pressure ventilation, awareness of the disease characteristics of PNP, a sufficient course of steroid therapy beforehand, and adequate mucosal protection are essential to the uneventful anesthetic management of the patient with PNP involving the respiratory tract mucosa.