Question 66 (part 2) · LDEK · Children with systemic diseases and disabilities

Presence of osteolytic defects in bones, lymph nodes enlargement, hepatosplenomegaly and oral cavity lesions in the form of gingivitis with tendency for ulcerations and granulation observed in a 10-year-old child may indicate:

  1. A. Chediak Higashi syndrome.
  2. B. Ehlers-Danlos syndrome.
  3. C. histiocytosis X.
  4. D. hypophosphatasia.
  5. E. Papillon-Lefevre syndrome.
Hint

Combine lytic bone lesions with lymphadenopathy and hepatosplenomegaly, and think of a proliferation of dendritic (antigen-presenting) cells.

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