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Dentistry · Oral pathology: cysts and odontogenic tumours: Langerhans cell histiocytosis
Sample itemA 3-year-old child has loose teeth that seem to float, gingival ulceration and punched-out radiolucencies of the alveolar bone. Biopsy shows cells with Birbeck granules on electron microscopy. What is the diagnosis?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
C. Langerhans cell histiocytosis
Langerhans cell histiocytosis is a clonal proliferation of CD1a-positive cells with Birbeck granules. Jaw involvement gives punched-out lesions and floating teeth, and multisystem disease can add skull lesions, diabetes insipidus and exophthalmos. Management depends on the extent.
Option by option
A. Hypophosphatasia
Hypophosphatasia causes early shedding with intact roots but no bone lesions or Birbeck granules.
B. Cherubism
Cherubism shows bilateral multilocular jaw expansion with a family pattern.
C. Langerhans cell histiocytosis (correct)
Correct. Birbeck granules with floating teeth and punched-out bone lesions define it.
D. Burkitt lymphoma
Burkitt lymphoma produces a rapidly growing jaw mass in a different age group with starry-sky histology.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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