Basal Cell Nevus Syndrome in a 9-Year-Old Boy

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Basal Cell Nevus Syndrome in a 9-Year-Old Boy

History and Clinical Presentation


A 9 year-old boy presents with a scalp lesion that he has had for 2 years.

History


This otherwise healthy white patient has had an asymptomatic scalp lesion on his scalp for 2 years. The child has a history of gross motor delay and low muscle tone, both of which have since resolved. He is an average student in the third grade. The patient's family has no history of genetic syndromes.

Physical Examination


On examination, the patient appeared to be in excellent general health. A 3- to 4-mm pink papule is seen on the right occipital scalp (Figure 1).



Figure 1. Scalp lesion.

The patient is also noted to have mild frontal bossing and macrocephaly. A biopsy of the scalp lesion was performed.

Histopathology




Figure 2. Low-power view of the biopsy specimen.



Figure 3. High-power view of the biopsy specimen.

A month later, the patient returned to the clinic with a second pink papule on the scalp. A biopsy showed histology similar to that of the first lesion.



Questions answered incorrectly will be highlighted.

What is the diagnosis?

Rombo syndrome

Gorlin syndrome

Brooke-Spiegler syndrome

Peutz-Jeghers syndrome

Cowden syndrome




Source...
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