The clinical spectrum of naevus anaemicus and its association with port wine stains: report of 15 cases and a review of the literature

Abstract

Fifteen patients with naevus anaemicus (NA) are reported. Twelve (8%) of 146 patients with a port wine stain (PWS) attending a laser clinic were noted to have this abnormality. In a control population of 298 new dermatology out-patient attenders, specifically examined for the presence of NA, three (1%) further cases were found. The clinical features of patients with NA are described and the literature reviewed. This is the largest group of patients with this disorder described in the English literature. The association of NA and PWS is confirmed. This study suggests that NA is more common than previously considered.

Nevus vascularis mixtus (cutaneous vascular twin nevi) associated with intracranial vascular malformation of the Dyke–Davidoff–Masson type in two patients

“…The vascular malformations recorded in the present patients are different from the spectrum of disorders including Sturge–Weber, Klippel–Trénaunay, and Parkes Weber disorders [Cohen et al, 2002; Ruggieri et al, 2008b]. The skin lesions in our patients were of the nevus anemicus and telangiectatic nevus types compared to the capillary (nevus flammeus), venous, arteriovenous, and lymphatic malformations seen in those disorders [Katugampola and Lanigan, 1996]. In addition, the two vascular nevi co‐occurred spatially and temporally and the brain lesions in the patients reported herein are consistent with the diagnosis of a Dyke–Davidoff–Masson malformation, rather than the vascular malformations of the leptomeningeal type as occurs in Sturge–Weber disease [Timura et al, 2005; Ruggieri et al, 2008a].…”

“…The vascular birthmarks (telangiectatic nevus and the nevus anemicus) present in these two patients may occur as an isolated skin disorder or as part of more complex malformation phenotypes [Katugampola and Lanigan, 1996; Sybert, 1997; Happle, 1999, 2002, 2005; Enjolras et al, 2007]. These have been explained by somatic mutations (loss of heterozygosity) occurring either at early developmental stages, thus giving rise to more complex mosaic skin and systemic disorders of the paired vascular nevi types, or at later embryonic stages giving rise to purely cutaneous traits [Happle, 1987, 1993a, 1993b, 1999, 2002, 2006; Koopman, 1999; Molho‐Pessach and Schaffer, 2011].…”

Nevus Anemicus: A Distinctive Cutaneous Finding in Neurofibromatosis Type 1

“…NA is rare in the general population but seems to have a much higher prevalence in patients with NF1. An association between NF1 and NA was first suggested in 1915 and was later confirmed .…”

Disorders of pigmentation

“…403 There is a predilection for the upper trunk, although involvement of the face, neck, and extremities occurs. 407 Onset in one patient was at age 21 years, suggesting the likelihood of an acquired variant of nevus anemicus. 405 Nevus anemicus sometimes occurs in association with neurofibromatosis, 406 phakomatosis pigmentovascularis, or port wine stains.…”