Papulosquamous disorders: IL36RN and generalised pustular psoriasis: 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 · Papulosquamous disorders: IL36RN and generalised pustular psoriasis

Sample item

A young woman with no previous psoriasis has recurrent episodes of fever with sheets of sterile pustules on erythematous skin, and a sibling is similarly affected. Loss-of-function mutation of the gene encoding which molecule is the best explanation?

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

The answer and the reasoning

B. IL-36 receptor antagonist

Biallelic loss-of-function mutations of IL36RN remove the brake on IL-36 signalling and cause familial generalised pustular psoriasis, sometimes called DITRA, often without plaque psoriasis. Blocking the IL-36 receptor is now a targeted treatment for flares. Other mutations such as CARD14 are described as well.

Option by option

  • A. Filaggrin

    Filaggrin mutations cause ichthyosis vulgaris and predispose to atopic dermatitis, not pustular flares.

  • B. IL-36 receptor antagonist (correct)

    Correct. IL36RN encodes the IL-36 receptor antagonist, and its loss drives sterile neutrophilic pustulosis.

  • C. Desmoglein 1

    Desmoglein 1 is targeted in pemphigus foliaceus and mutated in some palmoplantar keratodermas.

  • D. ABCA12 lipid transporter

    ABCA12 mutations cause harlequin ichthyosis in newborns.

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, IL-36 receptor antagonist. Biallelic loss-of-function mutations of IL36RN remove the brake on IL-36 signalling and cause familial generalised pustular psoriasis, sometimes called DITRA, often without plaque psoriasis.

Why is "Filaggrin" not the answer?

Filaggrin mutations cause ichthyosis vulgaris and predispose to atopic dermatitis, not pustular flares.

Why is "Desmoglein 1" not the answer?

Desmoglein 1 is targeted in pemphigus foliaceus and mutated in some palmoplantar keratodermas.

Why is "ABCA12 lipid transporter" not the answer?

ABCA12 mutations cause harlequin ichthyosis in newborns.

What concept does this question test?

It tests Papulosquamous disorders: IL36RN and generalised pustular psoriasis in Dermatology. Blocking the IL-36 receptor is now a targeted treatment for flares. Other mutations such as CARD14 are described as well.

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 Papulosquamous disorders: IL36RN and generalised pustular psoriasis?

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.