Rare mucosal lichen planus

Rare mucosal lichen planus is an uncommon variant of lichen planus that predominantly affects the mucosal surfaces of the body, including the oral mucosa, esophageal mucosa, genital mucosa, and occasionally other mucosal sites such as the conjunctiva, nasal passages, and larynx. Lichen planus is a chronic inflammatory condition mediated by T-lymphocytes that target the epithelial basal layer, leading to tissue damage and characteristic clinical findings. In the mucosal form, patients typically present with painful erosions, ulcerations, white reticular (lace-like) patches known as Wickham striae, erythematous areas, and desquamative lesions on affected mucosal surfaces. Oral involvement is the most common presentation, often affecting the buccal mucosa, tongue, and gingiva, and can significantly impair eating, drinking, and oral hygiene. The condition can be debilitating due to chronic pain, difficulty swallowing (when the esophagus is involved), and sexual dysfunction (when genital mucosa is affected). A particularly severe presentation involving multiple mucosal sites is sometimes referred to as vulvovaginal-gingival syndrome or plurimucosal lichen planus. Mucosal lichen planus carries a small but recognized risk of malignant transformation to squamous cell carcinoma, particularly in the oral cavity, necessitating long-term surveillance. The exact cause remains unclear, but it is considered an immune-mediated disorder with potential contributions from genetic susceptibility, environmental triggers, and immune dysregulation. Treatment is primarily aimed at controlling symptoms and reducing inflammation, as there is no definitive cure. First-line therapy typically includes topical corticosteroids applied to affected mucosal surfaces. For refractory or severe cases, topical calcineurin inhibitors (such as tacrolimus), systemic corticosteroids, or systemic immunosuppressive agents (such as mycophenolate mofetil, methotrexate, or azathioprine) may be employed. Regular follow-up with multidisciplinary care involving dermatologists, oral medicine specialists, gynecologists, and gastroenterologists is recommended depending on the sites involved.

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