NAEVUS VASCULOSUS

Synonyms.—Angioma; Nævus vascularis; Nævus sanguineus; Mother’s mark;
Birth-mark; Fr., Angiome; Nævus vasculaire; Ger., Angiome; Feuermal; Gefässmal.

Definition.—A congenital new growth and hypertrophy of the
vascular tissues of the corium and subcutaneous tissues, of a light red
to a deep bluish or purplish color, exceptionally making its appearance
a few weeks or later after birth.

Various divisions of the blood-vessel growths, or angiomata, are
made by different writers. Kaposi divides the cases into four classes:
(1) Telangiectasis; (2) vascular nævus; (3) angio-elephantiasis (also
called elephantiasis telangiectodes); (4) cavernous tumor. Unna²
makes a complete division between certain cases, which he designates
vascular moles (vascular nævi), both the flat and the elevated, from the
angiomata proper, the former being histologically primary angiectases,
without any capillary budding, consisting of dilatation of previously
existing vessels, and predominantly of the venous capillaries; angioma

¹ Besnier and Doyon, French translation of Kaposi’s treatise, vol. ii, p. 370.

² Unna, Histopathology, to whose article I am indebted.

NÆVUS VASCULOSUS

681

proper is characterized by both a new growth of capillaries, predominantly
the arterial capillaries, and dilatation. The former are in some forms
congenital and in others acquired, while the angiomata proper are mostly
congenital, but develop materially or mainly after birth. The latter
class is represented by the angioma simplex hyperplasticum of Virchow
(or the angioma plexiforme of Winiwarter, or the angioma simplex seu
glomeruliforme of Unna); and by the so-called cavernous angioma (angi-
oma cavernosum). The latter, excluding those examples now believed
to be partly or wholly lymphangiomatous in character, according to
Winiwarter,¹ is anatomically analogous to the corpora cavernosa, and
consists of soft tumors of lobular formation and semispheric or protruding
surface, and of a steely-blue, rarely a reddish, color. The simple an-
gioma consists of a variously sized, smooth, nodular or lumpy, compres­
sible growth, of a bluish-red to a bluish-black color, and is the common
angiomatous tumor (or, as more usually called, vascular nævus, capillary
nævus), noticed in infants chiefly about the head. As representing the
angiectases may be mentioned the telangiectases, consisting of capillary
dilatation, so common about the nose in acne rosacea; the papillary
capillary varices of old people, seen chiefly on the trunk, the vascular
nævi proper, of which an example is the so-called port-wine mark, and
finally the varicosities and cavernous changes observed in the veins of
the lower part of the legs.²

While these various distinctions and divisions are more scientifically
exact, to the clinician a description of the various conditions under the
two headings adopted by Duhring, Crocker, Hardaway, and others—
nævus vasculosus and telangiectasis—seems more satisfactory and suffi­
ciently comprehensive, and is the plan here followed, the former including
the congenital vascular new growths and all tumor-like formations, and
the latter the acquired capillary dilatations, with which may also be in­
cluded the others of Unna’s angiectases, excepting the vascular nævi
proper.

Symptoms.—One of the most common forms of the vascular
nævi encountered is that known as angioma simplex, angioma simplex
hyperplasticum, capillary nævus, etc., already briefly referred to, con­
sisting of red to bluish or purplish-red, slightly to considerably elevated,
usually readily compressible, growths observed in young infants. The
surface is either smooth, irregular, lumpy, or nodular; it may be smooth
at first, and then become subsequently uneven. It is of congenital origin,
although not infrequently at birth it is quite insignificant, and sometimes
scarcely perceptible, increasing rapidly in size in the first days or weeks
of life. It is most frequently seen about the head, either upon the scalp
or face, although it may also occur elsewhere. It is variable as to size—
from that of a bean to an area as large as the palm or greater. It re-

¹ Winiwarter, Die chirurgischen Krankheiten der Haut, 1892, p. 534.

² The so-called nœvus anœmicus might be mentioned here. Vörner in 1906, and
later Stein (Archiv, C. 1, April, 1910, p. 411), described a number of cases with this name
presenting one, several, or more scattered pale patches over various parts of the body
in which the skin was paler than the surrounding normal skin; due apparently to an
absence of development of the arteries and veins, their place being taken by capil­
laries; not infrequently there are associated telangiectatic nævi.

682

NEW GROWTHS

mains stationary or increases in extent, but usually, after reaching vari­
able dimensions, ceases to grow. In some instances, after a time,—
several months or longer,—retrogression takes place, the nævus becomes
gradually smaller, and finally disappears without trace or leaving a
slightly thinned looking or atrophic patch. In others the growth is,
unless treated, persistent. Anything that disturbs or impedes the circu­
lation of the part, as coughing, crying, position (gravity), leads to tem­
porary increased prominence. As a rule, it is somewhat spongy to the
touch, usually, however, quite soft and readily compressible; in other
cases comparatively firm. In exceptional instances, more especially
when involving a greater part of a region, as the ear or extremity, a firm
spongy character is noticed, connective-tissue increase being equally
present and of pronounced character,—the so-called angio-elephantiasis
elephantiasis telangiectodes, etc.,—in which, doubtless, too, in some cases
at least, there is also lyrnphangiomatous development (see also Elephan­
tiasis). Occasionally the surface is accidentally broken, or this takes
place spontaneously, and some hemorrhage results, sometimes of an ap­
parently dangerous character. Occasionally sloughing gradually ensues,
limiting itself to the nævus area, and this leads to cure, with slight scar­
ring. If the growth is a pronounced one, and especially when over bony
prominences, pulsation can usually be felt.

In occasional instances a nævus may undergo cystic or cavernous
changes, and it has been stated that it may possibly develop into the
angioma cavernosum of Winiwarter. This latter, a rare formation, is,
however, usually, and probably always, primary, arising commonly
in the first year of life, and in most instances having its start in a trauma,
even of a mild or insignificant character. Rarely is it congenital. It
may be diffused or defined, soft, lobulated, protruding, or hemispheric,
sometimes distinctly encapsulated. It is turgescent, often quite painful,
and tends to increase in size, in exceptional instances invading soft
tissues, cartilage, and even bone.

A form of nævus which is occasionally congenital, but usually ac­
quired, and therefore to be more especially referred to under Telangiec-
tasis (q. v.), is that known as nævus araneus, or spider nævus, consist­
ing of a red dot or spot with radiating red lines. A well-known, but
fortunately not very common, form of nævus is that known as the port-
wine mark, port-wine stain, claret stain, birth-mark, nævus flammeus,
nævus simplex (Feuermal of the Germans, and tache de feu of the
French). The terms angioma and angioma simplex are likewise occa­
sionally used to designate it. It is congenital, although in some in­
stances there is variable increase after birth. In size it varies from that
of a small, insignificant spot to several inches or more in diameter; and
exceptionally it may involve a whole region. The face is its common
site. It is rounded, ovalish, or irregular in shape, of a bright or dark-
red color, usually flattened, and often not perceptibly elevated. It may,
however, be raised above the surface, and present a smooth, uneven,
nodular surface, and sometimes with here and there verrucous-like
thickening or projections. Between this type and that first described,
angioma simplex or capillary nævus, all gradations are met with.

NÆVUS VASCULOSUS

683

To these several forms of nævi other terms are sometimes given,
when additional peculiarities or properties are present or associated.
Thus in some instances pulsations are quite distinct, and hence the term
pulsating nœvus; in others the color is dark, the blood-vessel growth
deep seated and chiefly venous,—venous nœvus, angioma varicosum,—
the surface is predominantly rough and tubercular,—nœvus tuberosus,
—slightly fungoidal or mulberry-like in appearance,—mulberry nœvus,
strawberry-mark (the latter also used with flat forms of strawberry color),
—and so on. Not only, however, may a nævus be turgescent and pul­
sating, but it may exceptionally be erectile, and rarely there is also more
or less hairy growth noticeable. In fact any or all constituents of the
integument may be participants along with the blood-vessel dilatation
and new growth.

As a rule but one nævus is present in a case, but occasionally there
may be two or three, and exceptionally, as in the remarkable instances
recorded by Ullmann,¹ Kopp,² Pollitzer,³ and Post,⁴ they may be numer­
ous and of wide distribution, those of the first two presenting some char­
acters of telangiectases. Besnier and Doyon⁵ are of the opinion that
generalized angiomatous or telangiectatic lesions are the forerunners or
first signs of malignant development, probably based upon the signifi­
cance of the early telangiectases observed in xeroderma pigmentosum.

Etiology and Pathology.—The cause of these blemishes is
not known. According to Gessler’s⁶ study of 1265 collated cases, the
affection is doubly as frequent in females as in males. Various factors
have been suggested, among which, more especially, are maternal im­
pressions and intra-uterine pressure, but neither will bear the scrutiny
of searching analysis, although as to the influence of maternal impres­
sions during pregnancy various striking instances are recorded, but even
in such the chances of pure coincidence or misinterpretation are so great
as to throw doubt upon relationship. Unna (loc. cit.) is a strong advo­
cate of the pressure theory, stating that “the almost entire limitation of
the congenital angiomata to the superficial layers would seem to point
out that they are developed by the action of some external cause.’’ His
clinical observations concerning this point have, he adds, shown him that
these growths are practically always on regions which are most likely
to suffer from pressure during intra-uterine life; and in support of this he

¹ Ullmann, Archiv, 1896, vol. xxxv, p. 195 (with case illustrations and histologic
cut; patient, a woman of forty-four; numerous bluish-red, small pea- to small hazel-nut-
sized growths on the face, coming out crop-like at irregular intervals; began apparently
as telangiectases; first appearance when verging on forty years).

² Kopp, ibid., 1897, vol. xxxviii, p. 69 (patient a young man aged nineteen; numer­
ous flat and nodular compressible lesions about genitalia and legs, and also appearing
on trunk and upper extremities; to some extent, especially in the flat lesions, of the na­
ture of telangiectases, and tending to bleed easily; began about puberty).

³ Pollitzer, Internat. Atlas, 1899, plate xlii (patient, male aged twenty-five; noticed
a few weeks after birth, and no change since; numerous, closely contiguous nævi, aver­
aging the size of a dime, over the entire surface, except head, palms, and soles).

⁴ Post, Jour. Cutan. Dis., 1903, p. 498 (with illustration).

⁵ Besnier and Doyon, French translation of Kaposi’s treatise, second ed., p. 357;
Campbell records (Jour. Amer. Med. Assoc, 1907, vol. xlviii, p. 2000) a case of venous
angioma of skin, showing beginning malignancy.

⁶ Gessler, Inaug. Dissertation, Tübingen, 1889, brief abs. in Monatshefie, 1890,
vol. x, p. 241.

684

NEW GROWTHS

states that “an extraordinary percentage (10 to 20 per cent.) of individ­
uals have a nævus in the neighborhood of the occipital fontanel, hidden
by the hair, though often only traces of it are to be found in adults.”
Out of 114 newborn infants examined by Pollitzer¹ for the purpose
of investigation of this point, 40, or 35 per cent., had nævi in this region.
These observations are more or less confirmatory of Depaul's² state­
ment, which has always seemed open to question, that nævi were found
in about one-third of the children born at the Paris Clinique, in most
of them, however, disappearing within a month. Gessler’s analysis
shows 76 per cent, about the head, 3 per cent, on the neck, 11 per cent,
on the trunk, and 9 per cent, on the extremities. It is not improbable,
therefore, that pressure may be an important factor, but it would seem
that Virchow’s belief, quoted by Unna (whose words I repeat), is more
probably the more influential one. “Virchow was the first to indicate a
possible anatomic cause, namely a connection of the embryonic fissures
of the skin, especially the branchial fissures, with the appearance of
angiomata at their areas of predilection (eyelids, cheeks, ears, nose,
lips), which he names the ‘fissural angiomata’; and, according to him,
‘a very slight irritative condition at the borders of these fissures, which
are very abundantly supplied with vessels, is sufficient to induce a greater
vascular development, which might possibly be recognized as a nævus,
but which remains latent and only later becomes manifest. ’ “

Anatomically vascular nævi have their seat principally in the papil­
lary layers of the corium. In some instances, however, the whole corium
as well as the hypoderm are involved. According to Billroth,³ the new
formation starts first from the capillary plexuses of the hair-follicles,
the sweat-glands, the sebaceous glands, or the fat-lobules. The growth
consists, according to its nature and development, of dilated as well as
newly formed blood-vessels, which may be but slightly, moderately,
or markedly dilated and abundant, in extreme instances reaching pouch-
like or cavernous distention and sinuses; in some lesions the process is
chiefly or wholly limited to the arterial capillaries, while in others (venous
nævi) the veins are predominantly implicated. Babes states that “in
many cases, however, the newly formed vessels correspond neither to
veins nor to capillaries, and form manifold convolutions and networks.”
In addition to the vascular dilatation and new growth, the connective
tissue, especially about the vessels, may be increased slightly or consid­
erably; in some instances, in fact, all tissues may participate. The
cavernous variety, as already remarked, according to Winiwarter, bears
some resemblance to the cavernous tissue of the penis.

Diagnosis and Prognosis.—These formations offer no dif­
ficulty as to recognition—they could scarcely be confused with other
lesions. The prognosis as to effect upon health or life is, of course,
wholly favorable, although exceptionally dangerous hemorrhage has
been noted in the elevated, growing capillary nævi in infants, but a fatal
outcome would certainly be a great rarity; in these instances, very usually

¹ Pollitzer, Bangs- Hardaway’s American Text-book, p. 1009.

² Depaul, quoted by Crocker, Diseases of the Skin, third ed., p. 962.

³ Billroth, quoted by Babes, Ziemssen’s Handbook of Skin Diseases, p. 601.

NÆVUS VASCULOSUS

685

from the pressure immediately made and the clotting which ensues,
retrogressive tendency is shown and the lesion may gradually disappear.
In an instance under my casual observation (not under my care), in­
volving the ear in an adult, which began in early life, there has been a
gradual aneurysm-like distention in late years, which, from its threat­
eningly dangerous character, has required surgical attention, the chief
supplying arteries being cut down upon and tied; temporary improve­
ment resulted, but the vascular dilatation, growth, distention, and tissue
thinning soon presented again, and a third operation, of tying the main
truncal artery, has recently been resorted to. In some cases of capillary
nævus, as already stated in describing it, there is not infrequently a
tendency, after a time, to undergo involution and to disappear, leaving
a faint atrophy or no trace at all; and this tendency is sometimes appar­
ently started by a slight knock or injury to the part or attempts at treat­
ment. Occasionally superficial ulcer ation ensues, and this is usually the
beginning of a spontaneous cure; the possibility of hemorrhage is to
be kept in view, but while this is sometimes temporarily alarming, it is
rarely dangerous. The firm, slightly elevated growth and the various
grades of the port-wine mark, as well as the larger cavernous nævi, are
persistent, although they seldom show any disposition to increase. In
the smaller capillary nævi and in the other circumscribed forms much
can usually be accomplished by treatment, and frequently a cure be
brought about; but in the large growths and in the port-wine varieties,
not much is to be expected.

Treatment.—The cases most commonly coming under dermato-
logic observation are those known as angioma simplex, or capillary
nævus, and the so-called port­ wine mark; the former being that type
brought for treatment during the first weeks or months of infantile
life, either because it is growing larger or simply as a blemish desirable
to be removed. In the last few years treatment by means of the appli­
cation of liquid air or carbon-dioxid snow (q. v.) has been warmly extolled;
I have used it (snow) satisfactorily in the angioma simplex type in infants
and young children; as yet I have had no opportunity with other types.
Wickham¹ has of late relied entirely on the use of radium for the removal
of this as well as other forms, and his results have certainly seemed satis­
factory.

While in the angioma simplex types liquid air and carbon-dioxid
snow method has largely supplanted other forms of treatment, the
latter are still resorted to, and are well worthy of continued notice.
The most satisfactory methods have heretofore been, according to my
experience, those of pressure, electrolysis, and puncturing with a needle
or sharpened stick charged with nitric acid. There can be no doubt
that in some of the cases but a slight impetus is needed to start the proc­
ess of involution and gradual disappearance, and, therefore, unless the
child has passed the age of one or two months, the mild plan should
always be tried. The simplest, and probably the best, of these to begin
with is that first named—pressure. This is effected by repeated and
thick paintings with collodion, continued for some days or a few weeks
¹ Wickham and Degrais, “Radiumtherapie,” Paris, 1909.

686

NEW GROWTHS

or longer. This pressure method can also be combined with discrete
puncturing, the latter being done in several places over the growth, \ to
\ inch apart, with an ordinary needle or, better, a triangular-edged
needle, slightly breaking up the tissues within, and then, with due aseptic
precautions, immediately applying the several coatings of collodion. The
“electric needle” can also be used in the same manner in conjunction
with pressure. The slight local disturbance so caused gives rise, when
pressure is continuously exerted, to more or less plastic exudation and
agglutination and gradual obliteration of the growth.

In many cases, however, the pressure plan, as thus outlined, is not
successful, and when the child has already passed the second or third
month, the growth seems to have become permanently established,
losing, as a rule, the disposition, often noticed earlier, to spontaneous
or easily provoked disappearance, and more energetic measures are nec­
essary, and which are also applicable at an earlier date when thought
preferable to the pressure plan. The two methods I have employed are
those of electrolysis and punctures by a needle charged with nitric acid.
Electrolysis, a method which has been favorably used by many, and
strongly advocated by Hardaway, Duhring, Fox, Jackson, and others,
answers well in some instances, as I also can testify; it occasionally brings
about a rapid result. The method¹ by electrolysis is not difficult,
although, as a rule, it is tedious, and must often be repeated, at intervals,
several or more times.

¹ In the smaller growths a current of from 1 to 3 or 4 milliampères is sufficient, but
in the larger and more pronounced formations a stronger current may be necessary. In
the former cases, and if the child is quite young and can be easily and firmly held, an
anesthetic is not necessary, but in older children and in extensive growths, electrolysis,
owing to the pain of the operation, cannot be satisfactorily managed without anes­
thesia. The needle, ordinarily in my practice, is attached to the negative electrode,
although others prefer the positive as more likely to bring about coagulation, and I
am not yet convinced which is the bettter plan; the other electrode with wet sponge or
cotton covering can be applied nearby, as on the neck or arm. In all electrolysis
operations about the head, especially the upper part, the current should be increased
gradually, and also broken off slowly, in order to avoid dizziness and other disagreeable
effects. When possible, needles can be attached to both electrodes and inserted in the
nævus. If attached to the positive electrode, the needle should be of gold or iridopla-
tinum, as a steel one undergoes oxidation (see Hypertrichosis). Some are in the habit
of coating the needle with rubber or other insulating substance, such as shellac, up to
within ¹/₈ inch of the point, in order to prevent action on the skin at the point of entrance.
The needle is inserted into the growth, preferably somewhat slantingly, and down to
the base, the current then allowed to act for one-half to two or three minutes; it is then
withdrawn and reintroduced at another part, and so the nævus gone over, the punctures
being ¹/₈ to ¹/₄ inch apart. They should not be made too close, lest too much surface
action ensue. If the growth is small, the needle can be introduced at the center, going
slantingly toward the side, allowed to act, then almost completely withdrawn, and then
thrust in another direction, after the manner of the “Marshall-Hall method,” and so
on. In some instances more influence is noted to result from the introduction of the
needle just at or outside of the edge of the growth, and thus going completely around
it at ¹/₈ to ¹/₄ inch intervals, encompassing the growth in this manner with the idea of
cutting over the basal vascular supply. In the larger growths treated by electrolysis
under anesthesia several needles can be attached to the electrode and inserted at differ­
ent points of the nævus. I have always preferred to do too little than too much at one
time, and then to repeat the treatment at intervals of one to three weeks; in this way
there is less risk of unnecessary destruction, and, moreover, in occasional instances a
trifling amount of such treatment wall start involution changes in the growth. When,
in the cases stated, anesthesia is necessary, as much as possible should, of course, be
done at the one time. The application of pressure for several hours or more after the
treatment is, I believe, of considerable value in aiding toward a good result.

NÆVUS VASCULOSUS                                  687

Instead of electrolysis, or conjointly with it, punctures with a needle
dipped in nitric acid can be employed; or the hard, smooth, sharp-pointed
wooden tooth­picks can also be used for this purpose. The needle or
tooth­pick is merely moistened with acid, and then gently and slowly
pressed into the growth from above, and the nævus thus gone over,
punctures being made about the same distance apart as in electrolysis.
As with this latter, frequent repetitions are sometimes necessary, and
subsequent pressure is of material advantage.

The removal of the so-called port-wine mark has been essayed from
time to time, and when the blemish is of small compass, much can be
done; but if at all of extensive area, the outlook is unpromising, and
usually the effort inadvisable. For the treatment of this blemish the
various plans already noted, except that of pressure alone, which would
be without influence, have been employed, in limited cases, with varying
results; unless done thoroughly enough to produce scarring, sometimes
more disfiguring than the original blemish, the amelioration or relief,
as a rule, scarcely justifies the trial. An exception to this, however, may
possibly be made in favor of the method by electrolysis as suggested and
practised by Hardaway,¹ and also favorably spoken of by Piffard, G. H.
Fox, and others, by which often a distinct lessening in the depth of the
color is attained. My experience has been about as Hardaway’s—occa­
sional partial and fairly satisfactory success, but usually, in my opinion,
scarcely sufficient amelioration to compensate for the trouble; in exten­
sive cases I should hesitate to advise it, certainly not without a clear and
candid statement to the patient as to its tediousness and chances of fail­
ure and at the most only partial success. Wickham relies upon radium.
Liquid air and carbon-dioxid snow have also been employed with mod­
erate success.

Among other plans of treatment of some of the varieties already
mentioned, as well as the deeper and more pronounced growths, may
be mentioned those by excision and galvanocautery, both of which have
been employed successfully in some instances. In a few rebellious cases
I have had recourse to puncturing with the galvanocautery needle,
combined with pressure, and found the plan of service. Well-defined
circumscribed growths could, as often practised by surgeons, be excised.
In larger nævi, excision, if practised, needs to be supplemented by the
Thiersch method of skin-grafting. The plan formerly much in vogue,
of injecting irritating liquids into the growth, needs to be mentioned only
to be condemned. Recently good results have been claimed from x-ray
exposures, pushed to the point of a moderate dermatitis. The high-
frequency current, with the point electrode, is also capable of lessening
the port-wine blemish, but not without some scarring.

In the smaller port-wine marks as well as the other highly-colored
forms an excellent method of concealment is by the use of a properly
tinted theatrical grease paint; the patient becomes skilled in its applica­
tion, so that the blemish can be pretty well masked.

¹ Hardaway, St. Louis Courier of Medicine, 1886, vol. xv, p. 201, and Trans. Amer.
Derm. Assoc, 1885, p. 18 (with discussion); also Morrow’s System, vol. iii (Derma­
tology), p. 498.

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