OEDEMA ANGIONEUROTICUM

Synonyms.—Acute non­inflammatory edema; Acute circumscribed edema; Angio-
neurotic edema; Œdema circumscriptum; Œdema cutis circumscriptum acutum;
Quincke‘s disease; Giant urticaria and Urticaria œdematosa (many cases); Fr., Œdème
aigu.

Definition.—An affection characterized by one, several, or more
acute circumscribed edematous swellings, usually in regions where the
tissues are readily distensible, as the eyelid, ear lobe, lip, etc. Quincke
was the first to call special attention to this somewhat rare and peculiar

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malady, since which time Jamieson, Strübing, Riehl, Collins, Osier, and
many others have reported cases.¹

Symptoms.—The swelling may present itself without consti­
tutional or other symptoms. In some instances, however, there are a
variable degree of premonitory malaise, gastro­intestinal disturbance,
and a feeling of being generally out of sorts, which usher in the cutaneous
phenomena and sometimes persist throughout the attack. Doubtless
in some instances these may be due to edematous swellings in the gastric
wall, in others to the development of toxins which provoke the malady.
The swelling itself, however, is acute, coming on suddenly, and reaching
full and usually enormous development in a few seconds or minutes.
Occasionally, however, it comes on somewhat gradually, one or two hours
elapsing before its acme is attained. The swelling is, as a rule, rather
sharply circumscribed; rarely with a diffusing tendency into the neigh­
boring tissues. If the eyelid is the region involved, the eye is usually
completely closed; if the lip, the part is stiff and large, and for the time
scarcely admits of opening; and if both lips are attacked, temporarily
incapacitating for talking or eating. The nose is also occasionally the
site of this swelling, and attains considerable dimensions, but not so large
as the lip, ear lobe, or eyelid, where the tissues permit of greater disten-
tion. The extremities are likewise frequently subject to these swellings.
Less commonly other parts may be attacked, even the tongue and
glottis. According to the analysis of 71 cases collected and tabulated by
Collins, the first attack was noted on the face in 29 instances, on the
extremities in 22, trunk 6, larynx 5, genitalia 3, stomach 3, gums and
palate 1, neck 1, and mastoid region 1. While the swelling is usually
enormous, in some cases it is relatively slight, or may vary somewhat
in different attacks. As a rule, but one part is swollen at one time,
although in some instances several lesions may appear simultaneously
or one after the other; it is more commonly shifting, disappearing in one
place and reappearing in another. The swelling may be of the normal
color of the skin or somewhat paler, or it may be of a pinkish or reddish
hue. It is somewhat hard, does not pit like ordinary edema, although a
slight depression of transitory character may usually be made by pressure.

The duration of the swelling varies; it may disappear as rapidly

¹ Some important literature references: Quincke, Monatshefte, 1882, p. 129; Jamie-
son, Edinburgh Med. Jour., June, 1883, p. 1090; J. E. Graham, Canadian Practitioner,
Feb., 1885, p. 33; Strübing, Zeitschr.f. klin. Med., 1885, p. 381 (with numerous refer­
ences); Matas, New Orleans Med. and Surg. Jour., 1887-88, vol. xv, p. 257; Riehl,
Wien. med. Presse, 1888, pp. 354, 398, and 431 (with references); Osier, Internat.
Jour. Med. Sci., 1888, p. 362; Elliot, Jour. Cutan. Dis., 1888, p. 19; Unna, Monats-
hefte, 1889, vol. viii, pp. 446 and 490; Hartzell, University Med. Magazine, May,
1890; Collins, Amer. Jour. Med. Sci., 1892, vol. civ, p. 654 (an admirable analytic
paper with full bibliography); E. W. Jacob, Brit. Jour. Derm., 1892, p. 155 (with bib­
liography); Schlesinger, Wien. klin. Wochenschr., 1898, p. 235; Wende, Jour. Cutan.
Dis., 1899, p. 178; Onuf (Onufrowicz), Med. Record, Aug. 5, 1899, p. 183 (and allied
conditions); Baruch, ibid., Aug. 19, 1899, p. 257; Kohn, American Medicine, Dec 21,
1901, p. 997 (with review of the literature and full bibliography); Morichaut-Beauchant,
Annales, 1906, p. 22 (review with many references); Burr, The Journal of Nervous and
Mental Diseases, July 12, 1912 (tongue chiefly, but at times other parts also); mercury
(patient had tertiary syphilitic symptoms) was thought at first to provoke or aggra­
vate; attacks ceased after administration of salvarsan; Wiel, Jour. Amer. Med.
Assoc, April 27, 1912, p. 1246 (5 cases, with brief review and references).

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as it came, or more commonly last several hours, or in some instances
a few days, and then rapidly or gradually melt away. The attack
may thus end, or it may continue by the appearance of one or more
swellings elsewhere, and persist for several days or one or two weeks;
exceptionally for months or a year or more, as in Graham's case,¹ the
patient scarcely being free from these evanescent swellings.

It is not uncommon for the malady to recur from time to time, and
it is believed that there is a tendency to recur at a point previously
involved, but this is probably merely due to the fact that there are
comparatively few regions at which it is prone to occur, rather than
to any engendered weakness of the part. In some cases there is itching
or burning, and almost always more or less tension or a feeling of stiff­
ness in the part occurs. The surface temperature in some cases has
been noted to be slightly elevated, in others reduced, and in others again
undisturbed. While some of the edematous swellings—one or several—
may be free from subjective symptoms, in others there is intense itching,
and there may also be here and there, scattered over the surface, ordinary
urticarial efflorescences. In some instances partial local anesthesia or
numbness has been noted for a short time after the disappearance of the
swelling.

Etiology and Pathology.—The affection is met with in both
sexes and almost at any age, being probably most frequent between
early youth and middle life. The manifestation is closely allied to
urticaria, as shown by the not infrequent association of ordinary wheals,
or history of urticarial attacks. Some cases, however, seem to be free
from suggestive subjective symptoms and other evidences of an urticarial
character. Strübing, Quincke, Osier, and others have called attention
to the frequently shown family predisposition. Osier in one instance
elicited a history of its occurrence in five generations. Various causes
are apparently exciting, the most frequent, as also observed in urticaria,
being articles of food which disturb digestion or give rise to the develop­
ment of gastric or intestinal toxins. Its most common subjects are found
in those of weak digestion and easily disturbed nervous system. In
Matas’ case of daily attack, the swellings seemed to take the place of a
malarial attack. In many instances there appears to be a systemic
sympathy or auto­intoxication; Lodor² states that all his cases except
one showed a marked malaise and depression preceding the attack, and
his patients could thus foretell the approaching outbreak. Horwitz³
observed 3 cases follow traumatism, and in Ashton‘s⁴ patients the attacks
followed exposure to draft or sudden cooling of the surface. The
outbreaks in Oppenheimer‘s patients were excited by alcoholic beverages.
Wende noted in the attacks in his case an association of albuminuria and
hemoglobinuria. Exceptionally aspirin has been noted to provoke a
condition, especially about the head and face, simulative of this malady.

Acute circumscribed edema is of angioneurotic origin—a vaso-
motor neurosis, in short, and similar to urticaria. It is probably true,

¹ E. E. Graham, Annals of Gynecology and Pediatry, April, 1894.

²Lodor, Medicine, Nov., 1898.              ³ Horwitz, Medical News, April 16, 1892.

⁴T. G. Ashton, Medical News, April 8, 1893.

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as Riehl concluded, that the exciting cause of these peripheral vasomotor
disturbances is to be found in the central nervous system. Lodor's
studies lead him to believe that the presence in the blood of a lympha-
gogue in pathologic quantity, and the rapid rise of lymph pressure,
produce, in areas of lessened resistance, a sudden and rapid vasomotor
paralysis in such regions. He quotes Heidenhain's observation as to the
various substances which, when injected into the circulation, have the
power of largely increasing the flow of lymph,—such food-products as
mussels, etc, sometimes egg-albumen, sugars, and the like,—and to
which often an attack is to be ascribed.

Diagnosis.—The features of angioneurotic edema—its usually
sudden appearance and disappearance, the absence of positive pitting
upon pressure, and the regions likely to be involved, with frequently
a history of former attacks or family tendency, and often of urticarial
outbreaks—are sufficiently characteristic to prevent error. Persistent
edema observed as a symptom in association with other diseases, with
lymphatic or glandular obstruction, is readily distinguished by its very
persistence.

Prognosis and Treatment.—The malady is usually a benign,
although often a troublesome, one. Rare exceptions, however, of
fatal termination due to involvement of larynx and glottis have been
observed. An immediate attack is generally amenable to treatment,
but freedom from future attacks cannot be assured. Much will depend
upon the patient's mode of living, freedom from nervous disturbance,
and strict attention to dietary. The management, in fact, is essentially
that of urticaria. Saline laxatives and antacids, more especially sodium
salicylate, have proved most useful in my hands, and Hartzell also has
had most satisfaction with this plan. Sodium benzoate in 5- to 20-grain
(0.35-1.35) doses, three times daily, can be used in place of sodium
salicylate, and for purgative or laxative purpose I can cordially commend
the ordinary antacid magnesia (see Urticaria). Salol as a gastro­intestinal
antiseptic is valuable. Arsenic, quinin, pilocarpin by subcutaneous
injection, atropin, ergot, bromids, etc., may also be mentioned, of which
the most valuable are pilocarpin and atropin. In Burr's case (a syphil­
itic) a dose of salvarsan had so far (some months later) resulted in
abolishing the attacks.

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