ABSTRACT
Paraneoplastic pemphigus (PNP) is a mucocutaneous blistering disease that is mostly fatal and is frequently induced by lymphoproliferative disorders. It can be considered as a paraneoplastic phenomenon that is caused by autoimmune disease but initiated by underlying lymphoproliferative disorder. The autoantibodies may deposit and damage skin, lungs, and other organs leading to the terminology paraneoplastic autoimmune multiorgan syndrome. PNP commonly affects adults between the ages of 45 and 70 years, though it has also been reported in children. Painful erosive mucositis is universally present, and a diagnosis of PNP should not be considered in its absence. Oral mucosal involvement is the initial manifestation in almost 50% of the patients. The buccal, labial, gingival, and lingual mucosae can all be involved. Cutaneous lesions usually develop after the onset of mucosal lesions. Multiple clinical variants have been described. Approximately 30%–40% of patients develop lung involvement, which is mostly fatal. Acantholysis of bronchial respiratory epithelium is demonstrable leading to restrictive bronchiolitis consistent with bronchiolitis obliterans, late in the course of the disease. The treatment of PNP includes treatment of underlying neoplasm, suppression of disease manifestations, and management of patient symptoms.
