REPORT OF A CASE
A 19-month-old boy with numerous nevi, including a 10-cm congenital nevus of the scalp, developed a pigmented left paraspinal plaque at the age of 10 months. There was hypertrichosis within the plaque, and the mother reported "bulging" when the area was stroked. A vertebral radiograph was normal. On physical examination, there was a 3x3-cm smooth, hyperpigmented plaque with hypertrichosis at approximately Level L2-4 in the left paraspinal region (Figure 1 and Figure 2). Magnetic resonance imaging of the lumbar spine was normal.
A skin biopsy specimen was obtained from the left paraspinal plaque (Figure 3 and Figure 4).
What is your diagnosis?
DIAGNOSIS:
Congenital smooth-muscle hamartoma.
HISTOPATHOLOGIC FINDINGS
The biopsy specimen demonstrated an unusual array of smooth-muscle bundles within the reticular dermis. These smooth-muscle bundles were well-defined and extended in various directions. Some of the smooth-muscle bundles appeared connected with hair follicles.
DISCUSSION
Congenital smooth-muscle hamartoma
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