Secondary Acquired Cholesteatoma in Langerhans Cell Histiocytosis Patient

Langerhans cell histiocytosis (LCH) is a multi-faceted disease defined by the accumulation of dendritic cells in various organs with characteristics similar to the epidermal Langerhans cells and can affect any organ of the body. It is most commonly seen in young adults and children. Cholesteatoma is a congenital or acquired condition and is categorized into primary and secondary cholesteatomas. Only a few reported cases of primary or secondary cholesteatoma have been reported among patients treated for temporal bone LCH. We report a case of secondary acquired cholesteatoma in a six-year-old girl after five years of her LCH treatment. The patient initially presented with ear discharge and aural polyp which did not improve with medical management. A computed tomography scan of the mastoid showed a left middle ear cavity and temporal bone lesion with bony erosions and total obliteration of the left external auditory canal. The patient underwent multiple biopsies, and a histopathological evaluation confirmed the diagnosis of cholesteatoma. The diagnosis of LCH can be difficult due to variable clinical manifestations. Involvement of the ear as resemblance to other diseases such as mastoiditis and chronic otitis media are quite common. Computed tomography scan and biopsy are reliable tools for diagnosis. Cholesteatoma following LCH remains a rare entity, hence, critical examination at follow-up visits is needed. Surgery remains the treatment of choice for cholesteatoma patients.