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Langerhans Cell Histiocytosis — RACP Paediatrics MCQ

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HardHaematology & OncologyLangerhans Cell HistiocytosisRACP Paediatrics

A 3-year-old boy presents with a bony mass on his skull, proptosis of the left eye, and a scaly rash resembling seborrhoeic dermatitis. Biopsy of the skull lesion shows Langerhans cells positive for CD1a and S100 with Birbeck granules on electron microscopy. What is the diagnosis?

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Correct answer: D — Langerhans cell histiocytosis

LCH presents with bony lytic lesions (skull, long bones), skin rash (resembling seborrhoeic dermatitis), diabetes insipidus, hepatosplenomegaly, and lymphadenopathy. CD1a positivity and Birbeck granules are diagnostic. Treatment ranges from observation for single-system disease to chemotherapy for multisystem involvement.

Reference: ANZCHOG – 2024 – Treatment Guidelines: LCH