Track: Case Reports

Abstract:

Hamartomas are tumor-like masses of haphazard tissue indigenous to the organ they originate in. Neurocristic cutaneous hamartomas (NCH) are benign skin lesions consisting of aberrant development of neuromesenchyme. These lesions are typically found in the head and neck region of children. We present a case of a 9-year-old female with alopecia of the right occipital scalp that had been present since birth and was interpreted clinically as aplasia cutis. The area was sensitive and caused the patient discomfort. Due to the patient’s symptoms, the lesion was excised. Histologic examination revealed epidermal acanthosis with prematurely attached sebaceous units to the overlying skin. The underlying dermis showed proliferation of neural structures with schwannian-like differentiation with tactoid bodies and surrounding bland spindle cells. The background hair follicles showed marked atrophy. Immunohistochemically, the neural bundles and tactoid bodies were diffusely immunoreactive with S-100 and SOX10. Surrounding spindle cells stained positively with EMA, confirming perineurium. CD34 was negative. This case is unique as NCH typically presents similarly to dermal nevi as a small lesion with minimal alopecia. Classically, the differential diagnosis for a congenital patch of alopecia includes aplasia cutis and nevus sebaceus; however, this case supports that NCH should not be excluded as a potential diagnosis.

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Emma Barnes