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THE

SPIDER

NEVUS

IN

INFANCY

AND

CHILDHOOD

James E. Wenzl, M.D., and E. Omer Burgert, Jr., M.D.

Section of Pediatrics, The Mayo Clinic, and Mayo Foundation, Rochester, Minnesota

(Submitted April 8; accepted for publication June 15, 1963.)

Dr. Wenzl is a Fellow in Pediatrics, Mayo Foundation.

PEDIATRICS, February 1964

227

T

HE spider nevus (nevus araneus) is a

vascular nevus composed of small telangiectases radiating from a central point in fine hairlike branches. The central point

or punctum is an artery; from it superficial blood vessels radiate peripherally. This unique vascular arrangement bears a super-ficial resemblance to a spider, hence the

descriptive term “spider nevus.” Since the central vessel is an artery and blood flow is centrifugal, gradually increasing pressure

over the central vessel will cause blanching of the lesion in a centripetal fashion. This

phenomenon may serve to differentiate

spider nevi from most Otiler telangiectases,

which generally blanch when pressure is

exerted over either end of the ectatic

blood vessel. The lesions of spider nevi may resemble hereditary hemorrhagic telangiec-tasia, but in the latter condition the

heredi-tary nature of the lesions, frequency of

hemorrhagic episodes, and presence of

telangiectatic vessels in the mucous

mern-branes make differentiation possible.

The spider nevus in adult patients has attracted much attention among physicians. Its association with hepatic disease and pregnancy is well documented. In addition, these lesions may be found in 10 to 15% of

normal adults.’ We know of no figures on the incidence of spider nevi in infants and children, although Bean’ has estimated that the incidence is approximately the same as among adults, that is, 10 to 15%.

Our interest in this subject was

stimu-lated by a 7-year-old girl who recently presented with tile complaint of idiopathic

hematunia. An extensive hematologic and

urologic work-up revealed nothing unusual to account for the hematuria; however, on physical examination eight spider nevi were

noted on the skin, and the question was

raised as to whether this was a significant clinical finding. Since no figures were

avail-able on the incidence of spider nevi in

children nor on how many might be seen

on a given “normal” child, we were stimu-hated to pursue the following study.

MATERIALS AND PROCEDURES

A total of 711 infants and children

rang-ing in age from 1 day to 15 years were

examined. The entire skin of each child was examined carefully for spider nevi. This

group was unselected, and each member

was seen by one of us during a 5-month

tenure on the diagnostic, well child, and hospital services at the Mayo Clinic. Care-ful records were kept on each child, includ-ing age, clinical diagnosis, associated skin

lesions, presence or absence of palmar

erythema, size of liver and spleen (by paipa-lion) and other pertinent data. If spider nevi were found, their anatomic location was recorded on an appropriate diagram on

the study card.

The clinical diagnoses encompassed a

wide variety of organic and functional ill-nesses. Approximately 2Z% of the total num-ben were regarded as being well children

(

seen for routine examination only). In 5% of the group a diagnosis of functional ill-ness was made. In approximately 30%, the

diagnosis was a surgical or primarily

surgi-cal problem. In the remaining 40%, the diag-nosis encompassed a wide variety of acute

and chronic illnesses.

For the purposes of this study we elected to divide the 711 children into three groups. We considered group 1 to be “normal” con-trols. This group included all the well chil-dren, children with functional illnesses, and children with acute infectious medical ill-nesses of less than 5 days’ duration. Also

included were patients having acute and

(2)

.lge

(yr)

0-S 190

6-10 104

11-13 113

Total 407

central nervous system, comprised group 2.

The conditions encountered in this group included congenital abnormalities, collagen diseases, metabolic and nutritional

disor-ders, hepatic disease, and chronic surgical problems. Patients with a history of

polio-myelitis were arbitrarily included in this group, as were those having peripheral neuritis, mvopathy, and meningitis.

Group 3 included those patients with dis-eases of the central nervous system, includ-ing all congenital abnormalities of this

sys-tem.

RESULTS

Group 1 (Normal Children)

GENERAL: The occurrence of spider nevi

according to age and sex distribution is

shown ill Table I. In 22% of the boys and

30% of the girls (all ages considered together

for eacil), one on more spider nevi were noted. The ages of increased incidence may he seen more clearly in Figure 1. After 2 years of age the incidence of spider nevi increases fairly rapidly (Fig. 1). It reaches

a plateau at approximately the age of pu-berty, and then appears to decrease among

adolescents after 12 to 13 years of age,

presumably toward the incidence among

adults of 10 to 15%. The incidence appears to increase more rapidly and earlier in girls

than in boys and to reach a higher plateau.

The occurrence of spider nevi appears to

increase among girls 8 to 15 years of age

The “t” test was applied to Table II for determining the significance of the differ-ences.#{176} For the total series the difference

between the two age groups (20.2 and

34.2%; 14.0 ± 4.45, p less than 0.01) was significant. For the difference between the sexes (22.1 and 30.1%; 8.0 ± 4.37) p equaled 0.06. However, for the group 8 to 15 years of age, the difference between the sexes (26.7 and 43.5%; 16.8 ± 7.67, p 0.03) was

significant. It is quite obvious that the dif-ference (2.2% = 21.5% - 19.3%) between the

two sexes in the age groups less than 8

years of age is not significant. In analyzing

the differences between the age groups by sex we find a significant difference for the girls (22.0 ± 7.08, p < 0.10), but not for the boys (7.4 ± 5.65, p = 0.19).

In group 1 a total of 199 spider nevi were found on 104 children for an incidence of 1.91 spider nevi per person. Of these 104 children, 51 were boys, on whom a total of 81 spider nevi were found (1.59 spider nevi

per person). On the 53 girls, a total of 118 spider nevi were found (2.23 spider nevi per person).

The greatest number of spider nevi found on a boy in this group was four; the great-est number on a girl was 11. In these two

children, the clinical diagnoses were “well

#{176}We are indebted to Mr. R. P. Gage, Section

of Biometry and Medical Statistics, for evaluation

of the significance of the data given in Tables II

and V.

TABLE I

AGE AN!) SEX: l)IsTIuBuTloN OF S1’IDF:lI Ni:vi IN NoIlst.L (‘IlIi.DIIEN

Boys and Girls Boy.’ (‘irls

J1it/i Spider \‘evi JJith Spider ‘e,’i JJ’itlz Spider Veri

Total

--

Total

-

Total

-Number Per cent \‘ui,iler Per rent Xii inber Per rent

14.7 109 15 13.8 81 13 16.1

39 37.5 58 17 9.3 46 47.8

37 37.7 64 19’ 9.7 49 18 36.7

(3)

Aqe,years

q)

E

13

-5

vs I::

.4.-vs

Fic. 1. The age-Sex distribution of spider nevi in normal children.

child” and “conversion reaction,” respec-tively.

ANATOMIC DISTRIBUTION: Most of the

spider nevi occurred over the dorsum of the hands and fingers, forearms, and facial areas (Table III). This pattern of

distribu-tion is at variance with Bean’s’ data con-cerning sites of spider nevi in adults. In his series, spider nevi occurred relatively

more frequently over the arms, neck and

thoracic areas both in normal adults and in

patients afflicted with hepatic disease.

The high incidence of occurrence over

the dorsum of the hands and fingers appears to be characteristic of the pediatric age group, as it was comparable in each of the three groups in our series.

DESCRIPTION OF INDIVIDUAL LESIONS: The essential elements of the spider nevi noted in this group were a body or central point

which was elevated in some but not in

others, the legs or radicals, and a

surround-TABLE II

Co%IPARISoN OF INCIDENCE OF SPIDER NEVI IN NORMAL Boys AND GulLs IN Two AGE Giioui’s

Boy.i and Girls

Age With Spider Nevi

(yr) Total

-Number Per cent

Boys Girls

Total

With Spider Neri

Number Per cent

Total

JJ7th Spider Neri

-Number Per cent

<8

8-13

252

153

51

53 34.2

143

86

28

23

19.3

26.7

107

69

23

30

21.5

43.5

(4)

i’:,-

-- ..

Fic. 2. Large spider nevus on facial area of a normal child.

Location Number Per cent

1)orsum of han(ls 78 39.2

Forearms 47 23.6

Face and ears 43 22.6

Arms H 6.0

Palnis (hands) 6 3.0

Lower extremities 4 2.0

Abdomen 3 1 .5

‘Fborax

Bk 2 1.0

Front 1 0.5

Neck 1 0.5

ing erythema. The erythema was inconstant and less apt to be seen with the smaller lesions. The size varied from several

milhi-meters to 2 cm or more in diameter. The

largest spider nevi seen were situated on the facial areas. Pulsations occasionally could be noted in the larger ones, but were

not detectable in the smaller ones. The

color ranged from a fiery red to lesser hues of the same color. A typical spider nevus

on the facial area of a normal child is shown

in Figure 2. No differences were noted

be-tween the anatomic characteristics of spider

nevi in tile control group as contrasted with those of tile otiler two groups.

EFFECT OF ACE: Spider nevi tended to occur less frequently in the first 4 years of

life; when present, however, they were

fre-quently large and prominent as compared to those seen ill older children. In this age

group they were found relatively oftener on

the inferior extremities, the abdomen and

PALMAR ERYTHEMA AND OTHER CUTANE. OUS ABNORMALITIES: Palmar erythema, of

the magnitude seen during pregnancy or in adults with cirrhosis, was not seen in this

group, although we specifically searched

for it. There was nO apparent association of spider nevi with other skin lesions.

Group 2 (Chronic Illness)

GENERAL: This group included 220

pa-tients. Tile age-sex distribution is

summar-ized ill Table IV. Although group 2 is

smaller than group 1, the age-sex

distribu-tiOll is approximately tile same. However,

there is one notable exception: in group 2 the increased incidence of spider nevi in girls more than 8 years of age as contrasted

with their male peers is not evident. In

fact, tile ratio is reversed. This discrepancy

may be a sampling error caused by the relatively smaller number of patients at

this age level, or it may reflect an

associa-tiOIl with diseases predominating in the males. If tile latter is true, it is not readily apparent from our analysis of the data.

A total of 65 spider nevi were found #{248}n

34 boys (1.91 spider nevi per patient); 55

spider nevi were noted on 25 girls (2.20 spider nevi per patient). These figures are similar to those in group 1.

The anatomic distribution was almost

identical to that seen in group 1, and the individual lesioiis vere of similar character.

DISEASE OF LIVER: Four patients in group

(5)

Age (yr)

---;i;---Boys and Girls Boys (,‘irl.

With Spider Ncri JVit/e Spider Xcvi

Total Total

46 Number 27 Number

Total

-;;---JJ’ith Spider Xeri

Number Percent 6-10 68 11-15 79 23 33 33.8 41.8 36 42 12 20 33.3 32 47.6 37 11 34.7 13 ‘35.1

Total 220 59 26.8 H4 34 27.4 96

TABLE IV

AGE AND SEX DISTRIBuTIoN OF SPIDER NEVI IN CHILDREN WITH Cimitoxic ILLNES.s

liver. A total of 19 spider nevi were found

on three of these patients. In the fourth patient, a girl, fibrocystic disease of the pancreas was manifested by cirrhosis, but tilere was no evidence of spider nevi. It may be of interest that only three spider nevi were found in the thoracic region in the total series of 711 patients, and two of these three occurred in patients with hepatic

disease.

Group 3 (Diseases of Central Nervous

System)

A total of 86 patients (all ages) were

diagnosed as having diseases of the central

nervous system which included various

types of seizure disorders, brain tumors,

hydrocephalus, microcephaly, mental and

motor retardation, and others. Patients hay-ing myopathies, peripheral neuritis,

men-ingitis and a ilistory of pohionlyelitis were

not included in this group. Of the 86

pa-tients, 34 (39.5%) were noted to have one or more spider nevi, as compared to an in-cidence of 25.6% in the normal children studied (Table V).

The incidence of spider nevi in tile chil-dren having diseases of the central nervous system was found to be significantly higher

than that found in the normal child (39.5

and l5.6%). The difference is 13.9 ± 5.33,

p < 0.01. For the group of children more than 4 years of age, the difference (16.1 ±

6.8, p = 0.02) also is significant. For the

children less than 4 years of age, the differ-ence obviously is not significant. In both

series the differences between the age

groups were significant (diseased 50.8 - 8.7 = 42.1 ± 11.9; normal 34.7 - 9.0 = 25.7 ± 4.52, p < 0.01). In the 34 patients with

dis-TABLE V

C0MP.uusoN OF INCIDENCE OF SPIDER NEvI IN Two AGE GROUPS OF NORMAL ChILDREN AND ChILDREN

WIT!! DISEASE OF TIlE CENTRAL NElivous SYSTEM

Group

Boys and Girls

Age

Less than 4 Years More than .4 l’ears

-Total

With Spider Neri

Z%umber Per cent

Total

With Spider Neci

Number Per cent

Total

With Spider Nevi

Number Per cent

(6)

nevi per patient). Again the anatomic

dis-tribution and individual lesions appeared similar to findings in group 1.

COMMENT: The incidence of spider nevi

in children was rather surprising to us. It

appears that these lesions, like many others, generally are unnoticed unless a specific search for them is made. From this study, it may be seen that the presence of spider nevi

in otllerwise healthy children is an

insignif-icant stigma.

The age-frequency distribution seen in

this series suggests that there may be a re-lationship between spider nevi and puberty, possibly through some as yet undefined hor-monal mechanism. The known association of spider nevi with pregnancy tends to

sup-port this concept, as does the apparent in-crease in spider nevi seen in normal puber-tal females in our series. Bean’ stated that he suspected a relationship between spider

nevi and estrogenic hormones but that he

had been unable to confirm it.

The apparent association between spider

nevi and disorders of the central nervous system is more difficult to explain. In this regard, at least three possibilities come to mind. First, this relationship may not be borne out in a larger series, a possibility, however, that seems unlikely. Second, there

may be some association with medications which these patients have received. This

hypothesis seems unlikely because a sizable

number of children in this group had not

received any specific medication. Third, there may be a direct relationship between

spider nevi and disease of tile central nerv-ous system per se. The latter choice would seem the most plausible explanation. The

various neurocutaneous syndromes are well known to pediatricians and neurologists. Studies elsewhere’ have shown a correlation

between the existence of pigmented nevi

and the diagnosis of epilepsy, even among those persons who did not have a family his-tory or other stigmas of neurofibromatosis.

The anatomic distribution of spider nevi in the area drained by the superior vena

propensity to occurrence over the periph-eral superior extremities, and the dorsum of the wrist and hand appears to be the site

of predilection. The occurrence over the

neck and thoracic areas is relatively less frequent among children than among adults. The reasons for these particular

relation-ships are not clear.

SUMMARY

At the Mayo Clinic 711 children, ranging in age from birth to 15 years, were

exam-med for the presence of spider nevi. The children were divided into three groups:

normal controls, patients with chronic ill-ness, and patients with disease of the cen-tral nervous system. In the normal children, the incidence of spider nevi increased

rap-idly after 2 years of age to reach a plateau at about the time of puberty for both sexes. In children more than 13 years of age the incidence appeared to be decreasing,

pre-sumably toward the stated incidence of 12 to 15% in so-called normal adults. In normal children, spider nevi appeared to occur more frequently in pubertal females than in pubertal males. The incidence was increased

in both sexes in patients more than 4 years of age with disease of the central nervous system. In I)atients with chronic illnesses,

there was no significant increase in spider nevi, but the ratio of pubertal females to pubertal males was reversed. The anatomic distribution ill all groups differed from that

of adults, tile dorsum of the hands and fore-arms being tile sites of predilection in

chil-dren. In view of the frequency of these

lesions in childhood, it appears that the presence of spider nevi in otherwise llealthy

children is an insignificant stigma.

REFERENCES

1. Bean, V. B.: Vascular Spiders and Related

Lesions of the Skin. Springfield, Illinois:

Charles C Thomas, Publisher, 1958, pp. 28,

51, 101-110.

2. Masland, R. L.: Personal communication to

(7)

1964;33;227

Pediatrics

James E. Wenzl and E. Omer Burgert, Jr.

THE SPIDER NEVUS IN INFANCY AND CHILDHOOD

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