Idiopathic lymphoplasmacellular mucositis of the lips: A case report and review of the literature

Abstract

Idiopathic lymphoplasmacellular mucositis (ILPM) is a group of disorders histologically characterized by dense infiltrates of lymphocytes and plasma cells in submucosa. These lesions were 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 who 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.

An Uncommon Case of Plasma Cell Mucositis of the Tongue in a Young Man

“…Several times, PCM lesions can enter into differential diagnosis with squamous cell carcinoma (SCC) [4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22]; in literature, there are cases in which a differential diagnosis was made with oral carcinoma and there is a case report where a SCC arose from a mucosal plasmacytosis [13]. Therefore, a correct diagnosis and adequate management of the disease are essential.…”

Lymphoplasmacellular mucositis ameliorated by α4β7 integrin inhibitor vedolizumab in a patient with ulcerative colitis

“…Treatment options include topical, intralesional, and systemic corticosteroids; topical and systemic antibiotics or antifungals; topical cyclosporine; topical tacrolimus; dapsone; isotretinoin; and liquid nitrogen cryotherapy 7 . Spontaneous regression over 6 months has also been observed 7 . Our patient's chronic course of ILPMD remained in remission while on vedolizumab and flared when her vedolizumab dosing was delayed.…”

“…No defined guidelines exist for the management of ILPMD. Treatment options include topical, intralesional, and systemic corticosteroids; topical and systemic antibiotics or antifungals; topical cyclosporine; topical tacrolimus; dapsone; isotretinoin; and liquid nitrogen cryotherapy 7 . Spontaneous regression over 6 months has also been observed 7 .…”

Cocaine‐induced plasma cell orificial mucositis

“…Since then, plasma-cell infiltrates have been found on other mucosal surfaces described with a variety of different terms, such as PCOM or idiopathic lymphoplasmacellular mucositis-dermatitis. 1 Although its aetiology is unknown, PCOM could represent an immune response to mechanical trauma or chronic irritants 1,2 (in this case, cocaine use). There is a slight predominance in middle age men (56.6 years).…”

“…Corticosteroids and the identification and elimination of the underlying stimulus seem to be effective in reducing plasma cell infiltration. 2 However, management may be challenging. Multiple treatments have been described without consistent efficacy: corticosteroids (topical, intralesional and systemic), other immunosuppressive agents, antibiotics, destruction techniques (liquid nitrogen, CO2 laser and electrocoagulation), excision and radiation therapy.…”