A pigmented hairy nevus with limb reduction

A 16-year-old boy presented with a brown flat lesion over the left upper back for 5–6 years. On examination, an ill-defined hyperpigmented patch of size 10 × 10 cm with hypertrichosis was present over the left scapular area and extending onto the left arm [Figure 1]. In addition, the left upper limb was shortened in length since birth and thoracic scoliosis was noted [Figure 2]. Hence, a diagnosis of Becker’s nevus syndrome (BNS) was made clinically and on investigating further, an X-ray spine showed thoracic scoliosis, rest being normal. The patient was counseled regarding the disease and referred to orthopedics for further management.

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Figure 1:

Clinical image of lesion over back showing ill-defined hyperpigmented patch of size 10 × 10 cm with hypertrichosis over the left scapular area and extending onto the left arm. In addition, the left upper limb is shortened in length with notable thoracic scoliosis.

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Figure 2:

Clinical image of patient showing shortening of the left upper limb and thoracic scoliosis.

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BNS is an epidermal nevus characterized by hyperpigmentation, hypertrichosis, and hamartomatous increase of smooth muscle fibers associated with various developmental defects including ipsilateral limb reduction as highlighted in this case.^[1]