Skin tumours: Brooke-Spiegler 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 · Skin tumours: Brooke-Spiegler syndrome

Sample item

Multiple scalp cylindromas forming a turban tumour and trichoepitheliomas on the face with autosomal dominant inheritance are due to a mutation in:

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

The answer and the reasoning

B. CYLD

Brooke-Spiegler syndrome is caused by CYLD tumour suppressor mutations and produces cylindromas, trichoepitheliomas and spiradenomas. Large scalp cylindromas give the turban tumour appearance. Surgical removal is used when lesions are symptomatic.

Option by option

  • A. FLCN

    FLCN mutations cause Birt-Hogg-Dube syndrome.

  • B. CYLD (correct)

    Correct. CYLD mutations cause Brooke-Spiegler syndrome.

  • C. PTEN

    PTEN mutations cause Cowden syndrome.

  • D. TSC1

    TSC1 mutations cause tuberous sclerosis.

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 B, CYLD. Brooke-Spiegler syndrome is caused by CYLD tumour suppressor mutations and produces cylindromas, trichoepitheliomas and spiradenomas.

Why is "FLCN" not the answer?

FLCN mutations cause Birt-Hogg-Dube syndrome.

Why is "PTEN" not the answer?

PTEN mutations cause Cowden syndrome.

Why is "TSC1" not the answer?

TSC1 mutations cause tuberous sclerosis.

What concept does this question test?

It tests Skin tumours: Brooke-Spiegler syndrome in Dermatology. Large scalp cylindromas give the turban tumour appearance. Surgical removal is used when lesions are symptomatic.

How hard is this question?

We tag it as an application question: it asks you to use a fact on a patient or a finding. 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 Skin tumours: Brooke-Spiegler 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.