Nevus Anemicus Clinical Presentation

Updated: Mar 08, 2019
  • Author: Loretta S 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.


Physical 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. Lesions typically are 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. [2, 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 vascularis mixtus or mixed vascular nevus describes the co-occurrence of nevus anemicus and vascular malformations of the reticular telangiectatic type; association with brain abnormalities of the Dyke-Davidoff-Masson type has been reported. [5] Nevus anemicus also has been described in patients with phakomatosis pigmentovascularis, a syndrome characterized by vascular and melanocytic nevi. [6, 7] Phakomatosis pigmentovascularis type IIa has been associated with primary choroidal melanoma. [8]