Premature loss of primary teeth, sharply marginated lucency, round cell infiltrate with numerous eosinophils

# A 12 year old patient presents with premature loss of primary teeth. On radiographic examination, a sharply marginated lucency is seen in the area of tooth loss. A biopsy specimen shows a round cell infiltrate with numerous eosinophils. Which of the following diagnosis is suggested?

A. Cherubism B. Gardener’s syndrome C. Fibrous dysplasia D. Langerhans’ cell disease

The correct answer is D. Langerhans’ cell disease.

Explanation: The clinical presentation of a 12-year-old patient with premature loss of primary teeth, a sharply marginated radiolucency on radiographic examination, and a biopsy showing a round cell infiltrate with numerous eosinophils strongly suggests Langerhans’ cell disease (also known as Langerhans cell histiocytosis, LCH). LCH is a rare disorder characterized by the proliferation of Langerhans cells, often affecting children. In the oral cavity, it commonly presents with premature tooth loss, "floating teeth" due to bone destruction, and sharply defined radiolucent lesions. The biopsy finding of a round cell infiltrate with eosinophils is characteristic, as LCH lesions often contain Langerhans cells (which appear as round cells) and a prominent eosinophilic infiltrate.

Explanation of other options:

• A. Cherubism: This is a genetic condition causing bilateral jaw swelling due to fibrous tissue replacement of bone, typically presenting with multilocular radiolucencies and a "cherubic" facial appearance. It is not typically associated with premature tooth loss or eosinophilic infiltrates.
• B. Gardner’s syndrome: This is a variant of familial adenomatous polyposis, associated with osteomas, supernumerary teeth, and colorectal polyps. It does not typically cause premature tooth loss or sharply marginated radiolucencies with eosinophilic infiltrates.
• C. Fibrous dysplasia: This condition involves the replacement of normal bone with fibrous tissue, leading to expansile, ground-glass radiopaque lesions rather than sharply marginated radiolucencies. It is not associated with eosinophilic infiltrates or premature tooth loss.

Thus, Langerhans’ cell disease best fits the described clinical, radiographic, and histologic findings.