ARTICLE
A macular naevus anaemicus is usually easy to diagnose. Rubbing the skin
causes erythema of the surrounding normal skin, whereas the naevus remains
pale and becomes more prominent. Further, the naevus anaemicus also usually
fails to react to vasodilatory substances and it can disappear after sympathic
blockade [1, 2]. Hypersensitivity to catecholamines has therefore been
suggested [1]. Interestingly in a patient with generalised dermatitis
the naevus area was spared from dermatitis [3]. We report here on a young
man who was referred to our clinic regarding a pale area on the chest
which extended as a band on the arm to the hand. We suspected a diagnosis
of macular and linear naevus anaemicus but a naevus depigmentosus could
not be completely ruled out. The patient was very concerned about the
pale macula on the chest, which he wanted to have treated with transplantation
as we do for patients with vitiligo and piebaldism [4].
Transplant studies with four punch grafts in a patient with naevus anaemicus
had previously demonstrated donor dominance [5]. We explained to the patient
that the thin sheets of skin that we use for treatment of leukoderma would
not help if the cause of the lesion was deeper in the dermis. Nevertheless
he wanted us to try a transplantation of the chest lesion, which we agreed
to perform. We used dermabrasion to remove the epidermis of the pale area,
which was then covered with thin sheets of autologous skin obtained by
an air-driven dermatome. Here we describe our findings before, during
and after operation.
Case report
The patient was a 20-year-old man, with a 180 cm2 large macula
with sharp but irregular borders, located on the left upper part of the
chest (Fig. 1). In the
pale area a few spots with teleangiectatic vessels could be seen. From
the chest lesion there was an approximately 2 cm broad linear extension
along the ulnar side of the arm to the hand. The line was sharply demarcated
with irregular borders, and seemed to be built up of small maculae (Fig.
2). The patient had been told that the areas had been there since
birth. When exposed to the sun, the pale areas became pigmented but to
a lesser extent than the surrounding skin. Diascopy on the somewhat tanned,
sun-exposed arm showed a decreased pigmentation of the pale band. Diascopy
on the non-sun exposed macula in the pectoral area showed the same colour
as the non-affected skin. Under Woods light the pale areas were not accentuated.
Rubbing of the skin also caused a slight immediate reddening of some parts
inside the pale areas. After about one minute the erythema in the affected
areas had disappeared, and these areas then contrasted markedly to the
surrounding skin reddening, which on the chest persisted for 3 hours and
on the arm for about 20 min. A biopsy specimen taken at another hospital
was reported to be normal. The patient was otherwise healthy and no similar
lesions were known in the family.
Transplantation
The recipient area was outlined with a black marker. Some reticulated
reddish areas within the macula became more apparent after the slight
stimulation of alcohol desinfection and outlining (Fig.
3). The macula was then anaesthetised with EMLA®
cream (Astra, Södertälje, SWEDEN) under plastic foil occlusion
for 2 hrs. When the cream was removed the skin was totally white in the
naevus region, due to the vasoconstriction caused by EMLA.
The epidermis and part of the papillary dermis was removed with a rotating
diamond wheel (19,000 rpm). The reticulated reddening, which we previously
had observed only in some spots, was now clearly visible in the whole
area as teleangiectatic dark-red vessels and broom-shaped capillaries
coming up from the dermis or stretching out horizontally (Fig.
4). The bleeding stopped immediately and a bluish colour was apparent,
which we do not see in patients undergoing dermabrasion in this region
for vitiligo, where small arterial bleedings normally continue for 5-15
min, giving the area a pale red colour.
A 0.1-0.2 mm thick sheet of skin was taken from the buttocks with a
Zimmer air dermatome. The denuded dermis at the donor site appeared normal.
The dermabraded area was covered with the skin and bandage was applied
for one week [4].
Examination of the transplant area after one year showed a naevus looking
exactly the same as before the transplantation, with no signs of change
in colour.
Discussion
The clinical picture of the patient's naevus, with a linear extension
on the arm, is unusual. We have found only one reported case [6]. However
segmental naevus anaemicus have been mentioned by Vörner [7], who
first described the condition and Graham Little [8]. Rubbing of the lesion
can cause an initial reddening of the pale area, if the mechanical stimulus
is strong. After some minutes it disappears and the typical contrast with
the surrounding skin becomes evident. Heat and certain drugs can also
cause a slight erythema in the naevus [5]. In our patient diascopy of
the sun-exposed tanned arm showed some pigmentation in the naevus area,
which was less than in the surrounding skin. This decrease in pigmentation
might be due to vasoconstriction during exposure to the sun, since the
tyrosinase needs oxygen to form melanin. In naevus depigmentosus, which
can be both macular and linear, the decreased pigmentation is seen also
during diascopy, but the affected area is usually more homogenous and
not mottled [9]. The fact that the linear band starts close to the macular
naevus anaemicus in our patient also favours the same diagnosis in the
two sites.
Naevus anaemicus has been described in association with macular teleangiectatic
naevi occurring in other areas or close to a port-wine stain [10-12].
Ham and Happle reviewed the literature and showed in 3 of their own cases
that the 2 types of naevi intermingled and therefore called it "naevus
vascularis mixtus". Later the concept of twin naevi was introduced which
could be explained by allelic somatic mutations [13]. The intermingling
of the components in twin spotting has later been further documented [14].
Koopman also showed that naevus anaemicus and teleangiectaticus can develop
in the same area [15]. He assumed that there exists a gene locus with
different alleles, controlling the balance between constriction and dilatation
of cutaneous blood vessels. Pairing and recombination of these chromosomes
in a postzygotic cell can exchange the segment bearing the allelic mutation.
As a result two genetically different daughter cells are formed, one homozygous
for vasoconstriction and one homozygous for vasodilatation. Thus one daughter
cell is the stem cell for the naevus anaemicus and the other for the naevus
teleangiectaticus. In our patient the abnormal-appearing teleangiectatic
vessels were located at the same site as the naevus anaemicus and would
have been missed without dermabrasion. Biopsy examinations from naevus
anaemicus lesions have shown a normal epidermis and no alteration in size
and structure of dermal blood vessels [5, 16]. Electron microscopy of
such specimens showed normal endothelial cells with capillary walls in
tight or almost complete apposition obscuring the majority of the lumina
[1, 16]. Since a biopsy specimen from our patient had also shown a normal
vessel structure, the vascular changes observed must have been functional
and therefore not detectable on routine histology. Biopsy specimens of
early port-wine stains display no vascular wall abnormalities, but with
advancing age the vessels become progressively dilated, with an increased
content of erythrocytes, changing the colour of the skin. The dilatation
occurs in the most superficial part of the dermis [17]. Smoller and Rosen
found an abnormal regulation of blood flow in port-wine stains, modulated
by neural mechanisms [18]. They used the S 100 protein to stain the nerves
and found that port-wine stains had significant decrease in nerve density
and increase in vessel-to-nerve ratio when compared to normal skin. Their
conclusions were confirmed by Gaylarde et al., who measured the
fall of transcutaneous pO2 after vasoconstrictor reflex response
to increased venous pressure on involved and uninvolved skin [19]. Studies
with vasodilating creams and a vasoconstricting corticosteroid containing
tape in port-wine stains also suggested a reduction in autonomic control
as an important etiological factor [20]. Another interesting report concerned
a 2 month old girl with a teleangiectatic naevus in the lumbar area which
at the age of 3 years had changed and showed a picture typical of naevus
anaemicus [21]. A similar development cannot be excluded in our patient.
As an explanation for the abnormally dilated
vessels in the denuded area in our patient, we believe that the very strong
mechanical and heat stimulus induced by dermabrasion caused the vessels
to overreact and appear abnormal. We want to emphasise that we have performed
dermabrasion in over 250 patients at different locations, including patients
with dermographism, but have never seen vessels of this type. We assume
that some teleangiectasis could have been present before the dermabrasion,
but it was only partly seen on account of lack of blood caused by the
arteriolar vasoconstriction.
CONCLUSION
Acknowledgement
This study was supported by a grant from the Åke Wiberg Foundation.
Article accepted on 27/06/01
REFERENCES
1. Greaves MW, Birkett D, Johnson C. Nevus anemicus: a unique
catecholamine-dependent nevus. Arch Dermatol 1970; 102: 172-6.
2. Fleisher TL, Zeligman I. Nevus anemicus. Arch Dermatol
1969; 100: 750-5.
3. Mizutani H, Ohyanagi S, Umeda Y, et al. Loss of cutaneous
delayed hypersensitivity reactions in nevus anemicus. Evidence for close
concordance of cutaneous delayed hypersensitivity and endothelial E-selectin
expression. Arch Dermatol 1997; 133: 617-20.
4. Olsson MJ, Juhlin L. Epidermal sheet grafts for repigmentation
of vitiligo and piebaldism, with a review of surgical techniques. Acta
Derm Venereol 1997; 77: 463-6.
5. Daniel RH, Hubler WR, Wolf JE, Holder WR. Nevus anemicus.
Donor-dominant defect. Arch Dermatol 1977; 113: 53-6.
6. Piorkowski FO. Systematisierte Gefässnaevi. Dermat
Zeitschr 1927; 51: 198-204.
7. Vörner H. Über naevus anaemicus. Arch Dermatol
Syphil (Berlin) 1906; 82: 391-8.
8. Graham Little EG. Case of naevus anaemicus (Vörner).
Br J Derm Syphil 1921; 33: 25-6.
9. Jimbow K Fitzpatrick TB, Szabo G, Hori Y. Congenital circumscribed
hypomelanosis: a characterization based on electron microscopic study
of tuberous sclerosis, nevus depigmentosus, and piebaldism. J Invest
Dermatol 1975; 64: 50-62.
10. Parkes Weber F, Harris KE. A case of widely-disturbed superficial
teleangiectatic naevus (capillary heamangiectatic naevus) associated with
areas of naevus anaemicus - indications that a portion of cerebral meninges
are similary involved. Br J Derm Syphil 1932; 44: 77-82.
11. Ham H, Happle R. Naevus vascularis mixtus. Hautartz
1986; 37: 388-92.
12. Katugampola GA, Lanigan SW. The clinical spectrum of naevus
anaemicus and its association with port wine stains: report of 15 cases
and a review of the literature. Br J Dermatol 1996; 134: 292-5.
13. Happle R. Allelic somatic mutations may explain vascular
twin nevi. Hum Genet 1991; 86: 321-2.
14. Happle R. Loss of heterozygosity in human skin. J Am Acad
Dermatol 1999; 41: 143-61.
15. Koopman RJJ. Concept of twin spotting. Am J Med Genet
1999; 85: 355-8.
16. Mountcastle EA, Diestelmeier MR, Lupton GP. Nevus anemicus.
J Am Acad Dermatol 1986; 14: 628-32.
17. Finley JL, Clark RA, Colvin RB, et al. Immunofluorescent
staining with antibodies to factor VIII, fibronectin, and collagenous
basement membrane protein in normal human skin and port wine stains. Arch
Dermatol 1982; 118: 971-5.
18. Smoller BR, Rosen S. Port-wine stains. A disease of altered
neural modulation of blood vessels? Arch Dermatol 1986; 122: 177-9.
19. Gaylarde PM, Dodd HJ, Sarkany I. Port-wine stains. Arch
Dermatol 1987; 123: 861-2.
20. Lanigan SW, Cotterill JA. Reduced vasoactive responses in
port wine stains. Br J Dermatol 1990; 122: 615-22.
21. Nurse DS. The evolution of cutis marmorata into naevus anaemicus.
Br J Dermatol 1973; 88: 204-5.
|
Version imprimable
