Temporal bone Langerhans cell histiocytosis with uterine cervix involvement and cutaneous Erdheim-Chester disease

With fewer than 40 documented cases, temporal bone Langerhans cell histiocytosis (LCH) is exceptionally rare in adults. We present a case of temporal bone and uterine cervix LCH in an adult with concurrent cutaneous Erdheim-Chester disease. A woman in her 50s presented with several months of unilateral right aural fullness, otalgia, otorrhoea and tinnitus, unresponsive to antibiotics and aural irrigation. CT of the temporal bone revealed right mastoid destruction, prompting further investigation. Histology obtained from mastoid exploration confirmed LCH. Initial management included radiotherapy, followed by methotrexate and later, cytarabine. Disease monitoring was conducted withPositron Emision Tomography-CT. Due to its rarity, LCH is often initially misdiagnosed. Clinicians should remain vigilant for LCH in patients presenting with unresolved otological symptoms that deviate from the expected clinical course. Swift communication between relevant medical teams is essential for rapid diagnosis and optimal management of potential multisystem LCH when symptoms present in different areas.