Nevus Anemicus Clinical Presentation

Updated: Jun 07, 2016
  • Author: Loretta Davis, MD; Chief Editor: William D James, MD  more...
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Patients with nevus anemicus typically present with an asymptomatic pale macule or patch that has been present since birth and grows with the child. Frequently, the lesion of nevus anemicus is noted as an incidental finding on skin examination.



Clinically, nevus anemicus is a circumscribed, rounded, oval or linear pale macule or patch with irregular margins that may be surrounded by satellite macules. Lesions may be single or multiple and may be located on any part of the body, but most lesions commonly are found on the upper chest. Nevus anemicus occurs more frequently in females and is usually asymptomatic. Nevus anemicus is noted at birth or in early childhood, although it may be easily overlooked. Stroking or rubbing of the surrounding skin can make subtle lesions more apparent.

Nevus anemicus usually persists unchanged throughout life. Lesions occur with increased frequency in patients with neurofibromatosis. [3, 4] Areas of nevus anemicus frequently are extensive and have been observed in close association with capillary malformations of port-wine stain type, a phenomenon attributed to somatic recombination. Nevus anemicus also has been described in patients with phakomatosis pigmentovascularis, a syndrome characterized by vascular and melanocytic nevi. [5, 6] Phakomatosis pigmentovascularis type IIa has been associated with primary choroidal melanoma. [7]



Nevus anemicus is due to a congenital anomaly of the cutaneous vasculature resulting in hypersensitivity to catecholamines, leading to localized vasoconstriction.