Idiopathic Lymphoplasmacellular Mucositis of the Lips: A Case Report and Review of the Literature

Idiopathic lymphoplasmacellular mucositis (ILPM) is a group of disorders histologically characterized by dense infiltrates of lymphocytes and plasma cells in submucosa. These lesions are initially observed on the glans penis and vulva, and later on buccal mucosa, lips, gingiva, tongue, epiglottis, larynx, and other mucosal surfaces have also been reported. We have reviewed the previously reported cases of ILPM with lip involvement, outlining the clinical presentation, treatment and outcome of the entity to date. The etiology of ILPM is unknown, and the diagnosis is largely one of exclusion. To date, no consensus or consistently effective treatment for ILPM is available. Herein, we report an 18-year-old woman presented with markedly swollen lips with severe erosions, bleeding and yellow to black crusts was diagnosed to have ILPM by histopathological study. The case occurred on rare lip site with an unusual appearance. Various treatment modalities were tried, but the responses were unsatisfactory and the lesion only responded to long term administration of oral steroids.