Mucous membrane pemphigoid (MMP) is a rare variant of the skin disease pemphigoid, which predominantly involves the mucous membranes. This rare autoimmune disease that infrequently affects the respiratory tract is characterized by subepithelial blister formation that may result in scarring. Immunopathologic examination of mucous membranes reveals the deposition of immunoglobulins and complement within the subepithelial basement membrane. We describe a patient with undiagnosed MMP, with a near-fatal presentation of central airway obstruction causing acute respiratory distress. The patient was successfully treated with emergent rigid bronchoscopic resection of a ball valve-like endotracheal mass, and diagnosed with a rare variant of pemphigoid disease, MMP. The patient was treated with mycophenolate and was clinically in remission, with bronchoscopically stable lesions at 1 year of follow-up.
Solitary fibrous tumor of the pleura (SFTP) is a rare tumor of fibroblastic origin. It can be quite vascular, and its surgical management carries the risk of a major intra-operative bleed. The pre-operative use of endobronchial ultrasound (EBUS) to visualize the vascular supply of the tumor has not been reported.We report a case of a patient presenting with progressive shortness of breath and cough who was found to have a very large pleural-based tumor. We describe the use of medical thoracoscopy and EBUS to establish the diagnosis of SFTP and to characterize the blood supply of the tumor.In the future, EBUS may provide an alternative to conventional angiography for both mapping and embolizing tumor blood supply.
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