Orthodontics: Papillon-Lefevre syndrome: practice question

A Dentistry question in the style of the NEET PG paper. Answer it first, then read why the right option is right and why each of the others is wrong.

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Dentistry · Orthodontics: Papillon-Lefevre syndrome

Sample item

A 5-year-old has thick scaly skin on the palms and soles and severe gingival inflammation with premature loss of primary teeth, then the permanent teeth. Which diagnosis is likely?

Original practice item, not a recalled exam question. Nothing here is recorded.

The answer and the reasoning

D. Papillon-Lefevre syndrome

Papillon-Lefevre syndrome is an autosomal recessive cathepsin C deficiency with palmoplantar keratoderma and early severe periodontitis. Teeth are lost successively in the primary and permanent dentitions.

Option by option

  • A. Cyclic neutropenia

    Cyclic neutropenia shows periodic fever and ulcers.

  • B. Hypophosphatasia

    Hypophosphatasia shows low alkaline phosphatase and loss of intact teeth, with no keratoderma.

  • C. Gorlin syndrome

    Gorlin syndrome shows keratocysts and basal cell carcinomas.

  • D. Papillon-Lefevre syndrome (correct)

    Correct. Keratoderma with early tooth loss defines the syndrome.

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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Questions about this Dentistry question

What is the answer to this Dentistry question?

The best answer is D, Papillon-Lefevre syndrome. Papillon-Lefevre syndrome is an autosomal recessive cathepsin C deficiency with palmoplantar keratoderma and early severe periodontitis.

Why is "Cyclic neutropenia" not the answer?

Cyclic neutropenia shows periodic fever and ulcers.

Why is "Hypophosphatasia" not the answer?

Hypophosphatasia shows low alkaline phosphatase and loss of intact teeth, with no keratoderma.

Why is "Gorlin syndrome" not the answer?

Gorlin syndrome shows keratocysts and basal cell carcinomas.

What concept does this question test?

It tests Orthodontics: Papillon-Lefevre syndrome in Dentistry. Teeth are lost successively in the primary and permanent dentitions.

How hard is this question?

We tag it as a multi-step question: it asks you to combine more than one idea. The tag is our own editorial judgement, not an official difficulty rating, so read the reason for every option, not only the answer.

How should I study Orthodontics: Papillon-Lefevre syndrome?

Learn the dental formula, eruption ages and tooth structure once, as tables. Then use the flashcards and the other Dentistry questions on this site to test what stuck.

Is this a real NEET PG question?

No. It is an original practice item (editorial status: sample, version 1). NextPrep does not publish recalled or leaked questions, and independent faculty review of this item has not been completed yet, so confirm important facts in a standard textbook.