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Langerhans cell histiocytosis (LCH) is an uncommon systemic disease characterized by the infiltration of one organ or more by Langerhans cells. Its clinical presentation is heterogeneous and depends on the affected organs. We report the rare case of a 43-year-old female presenting herself with central diabetes insipidus and receiving nasal desmopressin spray. Later, the patient developed multiple papules and pustules progressing to multiple ulcers affecting the retroauricular and axillary folds, as well as the oral and genital mucosas. The skin lesions had been evolving for several months. A histopathological examination of a skin biopsy confirmed the clinical suspicion of LCH.

Advanced ulcerative tumefactive lesions of the mucocutaneous surface in previously unrecognized Langerhans Cell Histiocytosis