LYMPHANGIOMA CIRCUMSCRIPTUM

Synonyms.—Lymphangioma cavernosum (Besnier); Lupus lymphaticus (Hutch-
inson); Lymphangiectodes; Lymphangioma simplex; Lymphangioma superficiale
simplex (Unna); Lymphangioma capillare varicosum (Török); Fr., Angiome cystique
(de Smet and Bock); Lymphangiome circonscrit vesiculeux (Brocq and Bernard).

Definition.—A limited, regional, or patch eruption connected
with the lymphatics, characterized by pin-head­ to small pea-sized,

¹ Thibiérge, Ikonographia Dermatologica, 1907, p. 69.

² Gottheil. Jour. Cutan. Dis., 1909, p. 277.

604

NEW GROWTHS

usually somewhat deep-seated, often red-dotted, closely crowded thick-
walled vesicles.

This rare disease, for which the name lymphangioma circumscrip-
tum, given by Morris, seems the most appropriate one, was first de­
scribed by Tilbury Fox, and later by Hutchinson, Köbner, Noyes and
Török, Morris, J. C. White, Leslie Roberts, Francis, Elliot, Hartzell,
Gilchrist, and others.¹

Symptoms.—The type of this rare malady is represented by one
or several contiguous or closely adjacent patches, composed of vari­
ously sized, thick-walled, frog-spawn-like, grayish, pinkish, or reddish
vesicles, somewhat thickly set or even slightly crowded or bunched.
If a single patch,—probably the most frequently observed,—it is usually
made up of two or three aggregations, with here and there a few discrete
vesicles between. The patch varies in size and shape, generally 1 to
3 or 4 inches in its largest diameter, andt rather irregularly rounded
or ovalish. The lesions, more especially the smaller and more recent
ones, present a glimmering, translucent, distinctly vesicular, grayish or
pearly aspect; in some cases some of the older lesions very often show
epithelial thickening and roughening, and the translucency is lost, and
when such a condition is predominant, a slightly warty appearance is
given to the individual elevations and to the patch as a whole. Quite
commonly, on the covering wall of the vesicle, minute telangiectases in
the form of dots or striæ are to be seen. This feature, if conspicuously

¹ Literature: Tilbury and Colcott Fox, London Pathol. Soc‘y Trans., 1879, voll. xxx,
p. 470 (with histology); Hutchinson, ibid., 1880, vol. xxxi, p. 342 (2 cases with colored
plates and histologic report by Sangster), and Arch. Surgery, 1889-90, vol. i, plates xv
and xvi (of above 2 cases and an additional one); Köbner, Virchow‘s Archiv, 1883,
vol. xciii, p. 343 (hand and arm, somewhat cavernous development; with 3 case
illustrations), also full translation in Annales, 1884, p. 293; Malcolm Morris, Inter­
national Atlas, 1889, plate i (colored illustration of his own case and Hutchinson‘s 3);
Noyes and Török, Brit. Jour. Derm., 1890, p. 359, and 1891, p. 8 (résumé and critical
review of cases (4 of which do not, however, come under this disease) to date; with
histologic examination, cuts, references); Török, Monatshefte, 1892, vol. xiv, p. 169
(relation to angiokeratoma—critical analysis of cases and principal references)—abs.
analysis in Brit. Jour. Derm., 1892, p. 397; Schmidt, Archiv, 1890, vol. xxii, p. 529 (2
cases, 1 of upper lip and oral mucous membrane; 2 histologic cuts; review and refer­
ences)—abs. analysis in Brit. Jour. Derm., 1892, p. 133; Jamieson, Edinburgh Med.
Jour., 1890, vol. xxxvi, p. 269 (case demonstration, with notes); Elliot, New York Med.
Record, 1891, vol. xxxix, p. 561; Besnier-Doyon, French translation of Kaposi, vol. ii,
p. 380; de Smet and Bock, Jour, de med. de chirurg. et de pharmacol. Bruxelles, 1891,
vol. xcii., p. 495; Hartzell, Medical News, 1892, Jan. 16 (with a résumé of 8 previously
reported cases and references); Epstein, Jour. Cutan. Dis., 1892, p. 213 (2 illustrations;
a somewhat anomalous case, seated about the genitalia, lower abdomen, and left
buttock, beginning when aged twenty-four, and tending to disappear); Francis, Brit.
Jour. Derm., 1893, pp. 33 and 65 (7 cases—1 or 2 not clearly defined, with résumé and
analysis of all previously reported cases); another case, ibid., p. 364; J. C. White, Jour.
Cutan. Dis., 1894, p. 474; Leslie Roberts, Brit. Jour. Derm., 1896, p. 309 (5 cases of
lymphangioma—various types); Gilchrist, Johns Hopkins Hosp. Bull., 1896, p. 138
(with histologic cut); Colcott Fox, Brit. Jour. Derm., 1896 (case demonstration);
Malcolm Morris, ibid., 1898, p. 52 (case demonstration); Walsh, ibid., p. 338 (case
demonstration—involving eye and eyelids); Freudweiler, Archiv, 1897, vol. xli, p. 323
(colored case illustration, histologic cuts, review, and references); Brocq and Bernard,
Annales, 1898, p. 305, “Sur le lymphangiome circonscrit de la peau et des muqueuses”
(an elaborate and exhaustive review of the whole subject, with résumé and references
and histologic cuts); Pawlof, Monatshefte, 1899, vol. xxix, p. 53 (with 2 histologic cuts,
and with review of histologic findings and references); Waelsch, Archiv, 1900, vol. li, p.
97 (with 2 colored plates and histologic review): Pollitzer, Jour. Cutan. Dis., 1906, p.
493 (2 cases, histologic with illustrations).

LYMPHANGIOMA CIRCUMSCRIPTUM                     665

developed, lends to the lesions a pinkish or pinkish-red, opalescent aspect,
and in some instances (Hutchinson) so marked as more or less completely
to mask their usual color. In some, from rupture of these minute capil­
lary vessels and admixture of the excaped blood,—usually minute in
quantity,—a deep-red, purplish, or blackish look is given the vesicles.
In a well-marked patch of long duration it is usual to find, therefore,
clear shining vesicles, vesicles capped with red dots or striæ, purplish or
blackish lesions, and wart-like elevations. The lesions are firm and, as
a rule, thick walled and not easily ruptured, although presenting a vesic­
ular appearance, which can readily be corroborated by pricking, the dis­
charge being slight, but sometimes leakage being continued for some
minutes or an hour or two. In occasional cases, as in White‘s patient,
there is, in places, crusting of very firm consistence, of a yellow or reddish
color, formed apparently by the coagulation of the contents of the vesicles,
and is quite tough and somewhat persistent.

In several instances (Besnier and Doyon, Hutchinson, J. C. White,
and others) the part and immediate vicinity have exhibited a recurring
erysipelatous inflammation, in all probability accidental, or possibly of
the same character as observed in other maladies with lymphatic in­
volvement. As a rule, there is but little if any distinct elevation of the
skin area in which the lesions are seated; in some cases, however, there
is an underlying nævoid, tumor-like elevation, and in others an under­
lying basis of lymphatic dilatation, and, on the extremities, a varicose
condition of the veins; these cases are somewhat questionable and anom­
alous, although the surface lesions and characters are identical. The
eruption may be on almost any part, but the shoulders, neck, and scapu­
lar region are favorite localities. According to Schmidt and Brocq and
Bernard, the lips and mouth may also be the seat of the malady. The
eruption is persistent, although some of the vesicles disappear, others
taking their place; and there may be some variation, but, as a rule, the
area is gradually extended. Occasionally, as in 1 (Hartzell's case) of
the 2 cases under my observation for some time, there was a gradual
shifting of the area, progressing at one side and receding at the other,
and, according to Hartzell, several years later the entire patch had
moved from the scapular region to the summit of the shoulder, the former
site showing some slight atrophy of the skin, faint pigmentation, and here
and there a few small, isolated papules. There are no subjective symp­
toms except those due to accidental circumstances.

Etiology.—With few exceptions the malady has begun in infancy
or early childhood, and it is quite probable that in most of them it was
congenital. It is observed in both sexes. In some cases it has been as­
sociated with nævi (Besnier and Doyon, Fox, Pye-Smith, and others).¹
In several instances lesions and lesional groups, apparently representing
this same malady, though possibly due to mechanical obstruction of the
lymphatics, have developed at the border of a scar following surgical
operation. Development—recurrences—at the border of previously
cauterized patches of the disease has also been noted.

¹ Pye-Smith, Diseases of the Skin, p. 359 (appearing upon a large congenital port-
wine-stain).

666

NEW GROWTHS

Pathology.—The histologic conditions have been investigated
by most of the observers already named (see literature). The process
has its seat more especially in the papillary and subpapillary layers of
the corium, and is now generally agreed to be of lymphatic origin. It
consists of lymphatic dilatation as well as new growth of these vessels,
resulting in somewhat flask- or funnel-shaped cavities. De Smet and
Bock take issue with this generally accepted conclusion and consider these
cavities or cysts to have their origin in the capillaries of the papillary
layer. Török believes that both the lymphatics and blood-vessels are
concerned in the process; mainly, however, the former. It would seem,
from a clinical standpoint, as well as from histologic findings by several
observers, that this has considerable basis, capillary dilatation and new
blood-vessel formation being quite pronounced in some instances,- al­
though practically absent in others. In fact, Besnier and de Smet and
Bock question the propriety of classing all the reported cases together,
believing that some are pseudo-lymphangiomata; not lymph vascular
growths at all, but true hemangiomata, in which the blood-cysts have
become filled with serum and converted into clear vesicles (Jacquet).
Gilchrist examined several differently sized lesions and found them all
to consist not only of dilated, but also hypertrophied, lymphatics of the
papillary (principally) and middle layers of the corium. Sangster‘s
investigations led him to believe that the deeper cavities are dilated
lymphatic channels, while those more superficially seated are to be as­
cribed to distention and rupture of the lymph-spaces in the papillary layer.
The cavities are often divided into several subdivisions by septa formed
of the unaltered corium, and a well-marked layer of cells can be traced,
forming an endothelial lining to the cavities (Bowen). Bowen also found
some infiltration of round-cells around the cysts and cavities in the earli­
est stage of the lesions, but none in other parts of the cutis, and Gilchrist
also noted collections of mononuclear cells in the corium. The epidermis
commonly shows but little change, in some places being slightly thinned,
in others thickened. The vesicular covering usually consists of the entire
epidermic layer, and sometimes a well-defined thin layer of connective
tissue; hence their firm and not readily ruptured character. The pig­
ment in the deep cells of the rete is frequently observed to be increased.
The contents of the cysts consists of very finely granular matter, lymph
coagula, a scanty, though variable number of leukocytes, and occasion­
ally a slight admixture of blood.

Diagnosis.—The character of the area, beginning usually in
early life and consisting of aggregated and crowded yellowish or grayish,
somewhat translucent, deep-seated, tough vesicles, some often with a
rough, thickened covering, and others with red dots or striæ, and occa­
sionally one, several, or more with purplish or blackish contents, are
sufficiently striking as to prevent confusion with any other malady.

Prognosis and Treatment.—There is but little, if any, tendency
to spontaneous disappearance, but, on the contrary, there is a disposition
to extend, although individual vesicles often disappear. Treatment
consists in thorough removal by cauterization, curet, or other means.
There is, however, a tendency to reappear at the edge of the scar, and

MULTIPLE, BENIGN, TUMOR-LIKE NEW GROWTHS 667

recurrence is almost a certainty if the removal has not been radically
complete. In a few instances electrolysis has been employed with a
favorable influence; each vesicle should be treated, and the whole area
gradually gone over.

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