Langerhans Cell Histiocytosis Involving The Brain, Lungs, Salivary Glands And Vulva: Report Of A Rare Case And Review Of Literature

Vulvar and salivary gland involvement are rare in Langerhans cell histiocytosis (LCH), for which less than 60 and 5 cases have been reported in the literature, respectively. We report a 22-year old woman with LCH and multisystem disease including involvement of the brain (diabetes insipidus), lungs (pneumothorax), salivary glands and vulva (skin lesion). Vulvar lesion included a 3×2 cm erythematous and ulcerated plaque on the fourchette and a 1×1 cm similar lesion on the right labia major. The lesions were slightly infiltrated and without any discharge. The diagnosis was confirmed by skin biopsies positive by immunohistochemistry for S100 and CD68. The skin lesion and the neck mass regressed completely with systemic chemotherapy. This case provides further support for using a combination of vinblastine and prednisolone in multisystem LCH.