Nevus simplex: A reconsideration of nomenclature, sites of involvement, and disease associations

BackgroundNevus simplex (NS) is a common birthmark on the forehead, glabella, upper eyelids, and nape. More widespread involvement can be confused with port-wine stains (nevus flammeus) and other vascular birthmarks.ObjectivesTo further categorize the anatomic locations in infants with extensive NS and evaluate for any possible disease associations.MethodsWe conducted a retrospective review of patients with extensive NS seen at two tertiary care centers.ResultsTwenty-seven patients with extensive NS were identified. All had at least one typical site of involvement: glabella (77.8%), nape (59.3%), and eyelids (55.6%). Additional sites were the scalp, including the vertex, occiput, parietal (66.7%); nose (66.7%); lip (59.2%); lumbosacral skin (55.6%); and upper and mid back (14.8 %).LimitationsRetrospective nature of the study and relatively small sample size.ConclusionsWe propose the term “nevus simplex complex” for NS with more widespread involvement beyond the typical sites. Consistent use of the term “nevus simplex” will aid in correct diagnosis and appropriate management of these birthmarks.