Puzzler poll: Urticarial rash in a 9-hours-old male

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Can you diagnose this patient in our most recent puzzler poll?

Image credit: Provided by case authors

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Image credit: Provided by case authors

Click to zoom in on image

Welcome to this Contemporary Pediatrics poll. Take a look at the following case below. After reading through the description, choose a multiple choice answer and try to guess the correct patient diagnosis.

Then, come back to ContemporaryPediatrics.com on Monday, February 24, 2025 at 10:00 PM ET or later for the full case presentation, differential diagnoses, and the correct patient diagnosis.

This case was provided by Irene Chern, MD, Aimee Johnson, MD, and Dana Toib, MD.

The case:

A 6-day-old male with history of waxing and waning urticarial rash presents to the general pediatrician’s office for evaluation. The parents describe the rash as red, raised skin lesions that become flat and congregate, lasting several hours to days, resolving without leaving marks and then appearing in new areas. The rash began shortly after birth, and it does not seem to bother the patient. The parents deny any other concerns, including vomiting, fever, joint swelling, oral ulcers, or irritability. The patient is breastfeeding well and has normal urination and bowel movements.

On review of the nursery notes, it appears that at 9-hours of life the patient developed a rash that started as small, blanching, erythematous, circular macules on the legs; gradually increasing in size and number; becoming raised and coalescing to large, diffuse patches on the legs, back, chest, face, and soles that resolved within several hours. The patient was born to a 30-year-old gravida one mother at 40-weeks gestation via spontaneous vaginal delivery. The parents are without consanguinity or family history of autoimmune or inflammatory diseases. The pregnancy was complicated by maternal hypothyroidism and group B Streptococcus positive status, with the mother adequately treated with levothyroxine and intrapartum antibiotics.

What is the diagnosis of this case, based on the information provided?

Cryopyrin Associated Periodic Syndrome
Neonatal Onset Multisystem Inflammatory Disease
Muckle Wells syndrome
None of the above

Visit the Contemporary Pediatrics website on Monday, February 24, 2025, at 10:00 AM ET or later for an article featuring the full case presentation, differential diagnosis, and correct patient diagnosis.

Thank you for taking this Contemporary Pediatrics poll!

For more puzzler case studies, click here.

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