Neurocutaneous syndromes: PHACE 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 · Neurocutaneous syndromes: PHACE syndrome

Sample item

A large segmental haemangioma of the face in an infant should prompt screening for which association?

APort-wine stain with glaucoma onlyBHypopigmented macules and seizuresCPosterior fossa malformation, arterial anomalies, cardiac defects such as coarctation, and eye abnormalitiesDLisch nodules and neurofibromas

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

The answer and the reasoning

C. Posterior fossa malformation, arterial anomalies, cardiac defects such as coarctation, and eye abnormalities

PHACE syndrome describes Posterior fossa malformations, large segmental facial Haemangioma, Arterial anomalies, Cardiac defects (especially coarctation) and Eye abnormalities. Infants with large facial segmental haemangiomas need MRI/MRA and echocardiography. Propranolol therapy requires cerebrovascular assessment.

Option by option

A. Port-wine stain with glaucoma only
Port-wine stain with glaucoma describes Sturge-Weber syndrome.
B. Hypopigmented macules and seizures
Hypopigmented macules with seizures suggest tuberous sclerosis.
C. Posterior fossa malformation, arterial anomalies, cardiac defects such as coarctation, and eye abnormalities (correct)
Correct. This is the PHACE syndrome spectrum.
D. Lisch nodules and neurofibromas
Lisch nodules and neurofibromas suggest NF1.

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.

Questions about this Dermatology question

What is the answer to this Dermatology question?

The best answer is C, Posterior fossa malformation, arterial anomalies, cardiac defects such as coarctation, and eye abnormalities. PHACE syndrome describes Posterior fossa malformations, large segmental facial Haemangioma, Arterial anomalies, Cardiac defects (especially coarctation) and Eye abnormalities.

Why is "Port-wine stain with glaucoma only" not the answer?

Port-wine stain with glaucoma describes Sturge-Weber syndrome.

Why is "Hypopigmented macules and seizures" not the answer?

Hypopigmented macules with seizures suggest tuberous sclerosis.

Why is "Lisch nodules and neurofibromas" not the answer?

Lisch nodules and neurofibromas suggest NF1.

What concept does this question test?

It tests Neurocutaneous syndromes: PHACE syndrome in Dermatology. Infants with large facial segmental haemangiomas need MRI/MRA and echocardiography. Propranolol therapy requires cerebrovascular assessment.

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 Neurocutaneous syndromes: PHACE 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.