LYMPHANGIOMA

661

LYMPHANGIOMA

The subject of lymphangioma remains still an obscure one, to which
the many conflicting case reports have as yet contributed but com­
paratively little definite knowledge. While, strictly speaking, this term
carries with it the implication of new growth of the lymphatics, yet in
some recorded instances there is more reason to believe that the condition
was one of lymphangiectasis. It is highly probable, however, that the
former does not exist without the latter, and that, therefore, in most
cases, there is an association of the two processes. A reading of the
literature shows that there are several varieties, some of which may be
classed as deep-seated cystic growths, others as more superficial lym­
phatic varicosities, consisting of more or less tortuous and crowded or
closely adjacent dilated, and possibly new, lymphatic channels, or dis­
crete, irregularly grouped or scattered, pea- to cherry-sized, tumor-like
dilatations; and still another in which the lesions are within the middle
and upper part of the corium, close to the surface, and crowded or
bunched together, forming plaques of pearly or pinkish-red, thick-walled
vesicles, often dotted with minute telangiectases or vascular tufts.
These several classes correspond to the divisions made by Wegner,¹
who placed the cases in three groups—simple lymphangioma, cystic
lymphangioma, and cavernous lymphangioma. Chipault‘s² classification
is based chiefly upon the part especially involved—whether affecting the
lymph-glands, the main lymphatic channels, or the plexuses, and with
further subdivisions, based upon the superficial or deep situation of the
process. It possibly is more in accord with histopathologic findings,
but is much more elaborate, and, considering our present knowledge,
probably unnecessarily so, and is certainly not so feasible or convenient
as that of Wegner. Most of the contributions are based upon the latter,
although the relationship of one form to another is not infrequently
observed, as shown in the contributions on the subject by Hoggan,³
Jarisch,⁴ Nasse,⁵ Leslie Roberts,⁶ and others.⁷

In some cases the lymphatic vesicular dilatation and dilated channels,
or lymphangiectasis, are not the consequence of a primary pathologic
process of these structures, but the result of mechanical injury and ob­
struction, as in the instances of Elliot,⁸ Besnier,⁹ and others. Lymphan-

¹ Wegner, Arch. f. klin. Chirurg., 1877, vol. xx, p. 641.

² Chipault, Gaz. des Hôp., 1888, p. 1329.

³ Hoggan, “Multiple Lymphatic Nævi of the Skin and Their Relations to Some
Kindred Diseases of the Lymphatics,” Jour. Anat. and Phys., 1884, vol. xxviii, p. 304
(with histologic cuts).

⁴ Jarisch, “Zur Lehre von den Hautgeschwülsten,” Archiv, 1894, vol. xxviii, p. 164
(with 15 histologic cuts and review of the subject and references).

⁵ Nasse, “Ueber Lymphangiome,” Arbeit, aus der chirurg. Klinik der Univ. Berlin
(Von Bergmann), fourth part, 1890, p. 1.

⁶ Leslie Roberts, “Five Cases of Lymphangioma,” Brit. Jour. Derm., 1896, p. 309
(with review and references).           ⁸ See literature of Lymphangioma circumscripturn­

⁷ Elliot, Jour. Cutan. Dis., 1894, p. 137 (vesicles developed at the edge of an old
scar, the manifestation apparently clinically similar to lympnangioma circumscriptum,
but histologic examination indicated dilatation of the lymphatic capillaries, probably
due to obstruction).

⁹ Besnier, quoted by Bowen, Twentieth Century Practice, vol. v (Diseases of the
Skin), p. 685 (an acute form of lymphangiectasis involving penis and scrotum, vesicles
and some edema developing eight days after a contusion of the penis).

662                                       NEW GROWTHS

giectasis is not only sometimes a result of demonstrable or probably
mechanical obstruction following injuries, but it is likewise a part of
certain maladies in which the same factor, while doubtless causative,
is not always so evident, as in elephantiasis. Some instances are also
observed in which, in a more or less limited region, there is distinct and
pronounced dilatation of the lymphatic vessels, forming elevated,
doughy-feeling, compressible, cord­ or knotted-chain-like vessels. Harda-
way¹ noted such a case in a woman in whom both thighs, the buttocks, and
lower part of the abdomen presented innumerable varices of the superficial
lymph-vessels. In a somewhat similar case under my own care, in a man
of thirty, the whole of one thigh, more especially on the inner and anterior
aspects, was the seat of a network of both superficial and deep-seated lym­
phatic dilatations, with here and there distinct cystic growths, forming
tumors partaking of the nature of both lymphangioma and fibroma;
the color of the covering skin is usually normal or slightly purplish.

Simple lymphangioma consists usually of both dilatation and new
growth, and doubtless the examples just cited, although also illustrating
lymphangiectasis, might be more properly considered as belonging to
this group. Ordinarily, however, the formation presents as isolated,
or adjacent circumscribed, compressible, and somewhat elastic swellings,
variously sized, and with sometimes dilated lymphatic channels leading
into them. They may be seen upon any part, but the genitalia and lips
and mouth are the most frequent localities. Not uncommonly there
may be some edema and thickening, and a condition of slight elephan­
tiasis results. The surface is sometimes scantily or moderately abun­
dantly beset with transparent vesicles, which, if ruptured or broken,
give exit to fluid exudation, occasionally a more or less prolonged leakage.
Bowen observed a case of “a boy of eighteen who presented upon the
inner side of the thigh a large, easily compressible tumor, which was
surrounded and covered by small vesicles," from which, when punctured,
“a milky fluid continued to exude for a long period, after which the large
tumor diminished greatly in size, but attained its former dimensions in
a short time.” Such lymphangiomata upon the lips usually give rise
to a condition designated macrocheilia, and when on the tongue, to macro-
glossia. Under simple lymphangioma should also be included, I believe,
the cases reported by Van Harlingen² and Pospelow,³ under the name
lymphangioma tuberosum cutis multiplex, with the belief that they repre­
sented the malady previously described by Kaposi.⁴ Leslie Roberts⁵
has since reported a similar case. Kaposi‘s case, and also a similar one
since described by Lesser and Beneke,⁶ belong, however, as now generally

¹ Hardaway, quoted by Hersman, Morrow‘s System, vol. iii, (Dermatology), p. 512.

² Van Harlingen, “A Case of Lymphangioma Tuberosum Cutis Multiplex,” Trans.
Amer. Derm. Assoc, 1881, p. 28 (abstract only—full paper never published).

³ Pospelow, “Ein Fall von Lymphangioma tuberosum cutis multiplex,” Archiv
1879, P. 521 (with colored case illustration and histologic cut).

⁴ Kaposi, Hebra and Kaposi, Hautkrankheiten, vol. ii, p. 282 (with histologic cuts).

⁵ Leslie Roberts, loc. cit. (case V).

⁶ Lesser and Beneke, Virchow's Arckiv, 1891, vol. cxxiii, p. 86 (with histologic cuts).
Heidingsfeld, “Lymphangioma Tuberosum Multiplex,” Jour. Cutan. Dis., 1908, p. 441,
reports a case typical of Kaposi‘s case, and discusses the classification of the various
similar and allied cases (with case illustration, histologic cuts, and bibliography).

LYMPHANGIOMA CIRCUMSCRIPTUM                     663

believed, to the increasing and somewhat confusing group of cases classed
under benign cystic epithelioma (q. v.), although Kaposi did not fully
concede this. In rare instances, as in the cases reported by Thibiérge¹
and Gottheil,² the lesions present some clinical resemblance to xanthoma
growths.

In the 3 cases of Van Harlingen and Pospelow and Roberts, repre­
senting apparently true lymphangiomatous development, the lesions
were somewhat numerous, scattered, varying in size from a pin-head to
a hazel-nut, and elastic and compressible; the integumental covering
was apparently normal, although mostly of a rosy or a violaceous tinge;
some lesions had a pale violaceous or bluish, translucent-looking center.
Associated with these formations was a somewhat dingy condition of the
skin and spots or areas of pigmentation. A few of the tumors may re­
semble small, flabby, molluscum fibrosum growths, but for the most
part they are smooth, rounded, or ovoidal elevations, and so compressible
under the finger as to feel like “bladders filled with air and to give the
sensation similar to that of an umbilical hernia in a child.” They were
free from inflammatory action, and there were no subjective symptoms.
The three patients were women, aged twenty-three, thirty, and thirty-
two. Microscopic examination showed the structure to be composed
of fibrous and granulation-cell tissue, with numerous irregular spaces—
sections of dilated lymphatic vessels (Van Harlingen).

Cystic lymphangioma, another of Wegner‘s classes, needs to be but
cursorily referred to here, as it rarely comes under the observation of
the dermatologist, belonging essentially to the province of surgery, to
the works on which the reader is referred for a descriptive account. It
is usually congenital in origin, consisting of large multilocular cysts,
most commonly seen on the upper part of the neck, in which region they
are often known as hygromata colli. In this locality their prolongation
may extend somewhat deeply, going in between the muscles, even as far
as the mediastinum (Bowen).

Cavernous lymphangioma, the other group in Wegner‘s classification,
as it is commonly observed in the domain of dermatologic practice is
that form of lymphangioma to which the name of lymphangioma cir-
cumscriptum is given, and which is, therefore, owing to its importance,
given separate description. While it has in its purest type well-defined
and fairly uniform clinical characteristics, it presents in some instances,
as an analytic study of the cases reported will show, features indicating
a relationship to other types of lymphangioma and also to nævoid forma­
tions.

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