Vesiculobullous disorders: Kindler syndrome: practice question

A Dermatology 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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Dermatology · Vesiculobullous disorders: Kindler syndrome

Sample item

An infant has acral blistering that heals into poikiloderma over the years, with photosensitivity, fragile gums and webbing of the fingers. Which gene is mutated?

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

The answer and the reasoning

A. FERMT1

Kindler syndrome is an autosomal recessive genodermatosis from FERMT1 mutations, encoding kindlin-1. It combines blistering in infancy with later poikiloderma, photosensitivity and mucosal fragility.

Option by option

  • A. FERMT1 (correct)

    Correct. FERMT1 (kindlin-1) mutations cause Kindler syndrome.

  • B. LAMB3

    LAMB3 mutations cause junctional epidermolysis bullosa.

  • C. KRT14

    KRT14 mutations cause epidermolysis bullosa simplex without poikiloderma.

  • D. COL7A1

    COL7A1 mutations cause dystrophic epidermolysis bullosa with scarring and nail loss.

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 Dermatology question

What is the answer to this Dermatology question?

The best answer is A, FERMT1. Kindler syndrome is an autosomal recessive genodermatosis from FERMT1 mutations, encoding kindlin-1.

Why is "LAMB3" not the answer?

LAMB3 mutations cause junctional epidermolysis bullosa.

Why is "KRT14" not the answer?

KRT14 mutations cause epidermolysis bullosa simplex without poikiloderma.

Why is "COL7A1" not the answer?

COL7A1 mutations cause dystrophic epidermolysis bullosa with scarring and nail loss.

What concept does this question test?

It tests Vesiculobullous disorders: Kindler syndrome in Dermatology. It combines blistering in infancy with later poikiloderma, photosensitivity and mucosal fragility.

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 Vesiculobullous disorders: Kindler syndrome?

Learn each condition by its primary lesion, site and signature sign. Then use the flashcards and the other Dermatology 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.