Race for the Case

Race for the Case is a clinical quiz feature published quarterly in DermWorld Directions in Residency — and right here!

Answer the questions below to enter. Looking for the Fall 2022 answers? Review them now. To submit cases and questions, email Dean Monti at dmonti@aad.org.

Diagnose this case by Eduardo Michelen Gómez, MD and Marely Santiago-Vázquez, MD, FAAD

A 23-day-old baby girl was born at term via spontaneous vaginal delivery to a healthy G1P1A0 mother. The mother consulted to dermatology services for evaluation of linear vesicular eruption in left inner thigh and left mid calf. The mother noticed the lesions shortly after birth as solitary vesicular lesions involving the left medial inner thigh, with eventual progression to linear vesicular eruption. HSV PCR, IgG, and IgM were unremarkable. The mother denied any personal or known history of similar eruptions, painful vesicular eruption, previous history of STDs, or family history of similar eruptions. She also does not report any history of hypopigmentation, hair loss, or tooth loss at early age, and no prenatal or intrapartum complications. Mother received adequate prenatal care.

1. What is the most likely diagnosis and most common mutation?

2. Describe the clinical and pathologic findings of the different stages of this genodermatoses.

3. Which genetic condition is associated with the typical clinical presentation of this condition in male patients?

4. What are the most common extracutaneous findings associated with this genodermatoses?

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