Nevus anaemicus Q82.5

Congenital, usually bizarre and irregular (broken up outline), limited, bright, symptom-free spot, which is caused by permanent constriction of the dermal vessels (hypersensitivity to catecholamines) and is usually discovered later in life by chance.

• Unknown, a disordered function of motor end plates of smooth vascular muscles caused by the disposition or an increased endogenous sensitivity of skin vessels to catecholamines is discussed.
• There is a high association between nevus anaemicus and neurofibromatosis type I. In > 50% of the patients with neurofibromatosis type I a N. anaemicus is found in addition to the typical café-au-lait spots gfls. together with juvenile xanthogranulomas.
• There are also known individual cases that describe a coincidence with tuberous sclerosis.

Chest area, extremities

Coin- to palm size also larger, at the edges almost jagged, white, single or multiple count stain.

This bizarre stain is visible with varying degrees of clarity depending on the state of circulation in the surrounding area. It is particularly noticeable as a "negative contrast" when the surroundings are reddened. After intensive rubbing of the stain, in contrast to the "healthy" surroundings, no reddening of the affected skin is visible.

The boundaries to the environment disappear when printed with a glass spatula.

No vascular changes. Therefore the findings are normal.

Incontinentia pigmenti achromians: Pigmentary changes follow the Blaschko lines

Vitiligo: Acquired, migrating, borders to healthy skin not dissected, areas turn red on the rubbing test (hyperemia)

Naevus depigmentosus: congenital, pigmentary changes follow the Blaschko lines; areas turn red in the rubbing test (hyperemia)

Extragenital Lichen sclerosus: confetti-like, small-hearth distribution pattern, surface glossy.

The topical encounter of a nevus anemicus with a nevus flammeus is observed in rare cases and is called vasculartwin nevus or vascularis mixtus.

1. Ferrari F et al (2014) Juvenile xanthogranuloma and nevus anemicus in the diagnosis of neurofibromatosis type 1 JAMA Dermatol 150:42-46
2. Hamm H et al (1986) Naevus vascularis mixtus. dermatologist 37: 388-392
3. Hernández-Martín A et al (2015) Nevus Anemicus: A Distinctive Cutaneous Finding in Neurofibromatosis Type 1 Pediatr Dermatol doi: 10.1111/pde.12525
4. Juhlin L et al (2001) Naevus anaemicus with teleangiectatic vessels. Eur J Dermatol 11: 518-520
5. Marque M et al (2013) Nevus anemicus in neurofibromatosis type 1: a potential newdiagnostic criterion. J Am Acad Dermatol 69:768-775
6. Plantin P, Schoenlaub P (2001) Multiple anemic macules on the arms: not a variant form of nevus anemicus. Dermatology 202: 271-272
7. Sachs C et al. (2015) Nevus Anemicus and Bier spots in Tuberous Sclerosis Complex. JAMA Dermatol 18:1-2
8. Sarifakioglu E et al (2006) Multiple anaemic macules of the arms: a variant of Bier's spots or naevus anemicus? J Eur Acad Dermatol Venereol 20:892-893
9. Vörner H (1906) About Naevus anaemicus. Arch Dermatol Syphilis (Vienna) 82: 391-398