Langerhans cell histiocytosis (LCH) is a rare sporadic disease characterized by histiocytic
neoplastic infiltration of various organ systems and a wide spectrum of clinical
manifestations, ranging from benign and self-limiting to lethal. Herein, we report a rare case
of adult-onset multi-systemic LCH in a 36-year-old male patient with an initial perianal
presentation and incidental finding of subsequent thyroid gland involvement in the follow-up
period. The patient with a history of perianal LCH treated with surgical excision and local …

Conclusion: Our findings underscore the importance of awareness of this rare condition
associated with low index of suspicion among practitioners for LCH as well as the
significance of ensuring proper follow-up in adult onset LCH to rule out systemic disease.
Along with implementing a treatment plan matching the prognostic stratification of the
patient, multidisciplinary approach and long-term close follow up with PET/CT scan in the
management of adult onset LCH seem crucial given its rarity and varied presentation.