ACUTE CIECUMSCRIBMtJffiDIMA: 
By Rudolph Matas, M. D. '■ / 
Read before the Orleans Parish Medical Society. 
It was in the month of May, I believe, in 1886, when I 
was called to attend a Mrs. V. G., an Italian woman, aged 
thirty-six, who complained to me of the symptoms which 
constitute the basis of this narrative. Before proceeding 
with the details of her case, however, I will state that she 
had been nry client several years previously, during which 
time I had on several occasions attended to her husband 
and her children (five in number) for various ailments, 
among which, however, predominated the disorders due 
to malarial causation. The patient herself enjoys an 
average good health, though she somewhat exemplifies, 
in her dark, sallow complexion, etc., the “tropic diathe- 
sis” so ably described by our distinguished fellow mem- 
ber, Prof, Elliott, and which is so commonly met with 
among the laboring classes of our Italian population. In 
justice to her health, however, I must state that, outside of 
malarial fevers and a few accidental complications of 
childbirth, she rarely suffered with disease prior to the oc- 
casion when she called me to relieve her of the condition 
now under consideration. When I reached her house, 
which was late in the evening of the day that I had been 
sent for, I found her apparently as well as I had ever seen 
her and attending to her usual avocations. As I made 
some remark to the effect that she appeared too well to 
require medical assistance, and expressed some surprise at 
her sending for me, she told me that if I had only called a 
few hours earlier I would not have thought that she looked 
so well. She was feeling now, as usual in the evening, 
quite well; but it was “quite a different thing in the 
morning.” In fact, for the last two months and over, she 
had been afflicted with a peculiar disfigurement of the face, 
which “ could hardly be designated as sickness,” for it 2 
seemed, to her, too much of a trifle in the beginning to be 
worthy of such a name, but now she was becoming 
alarmed as it persisted so obstinately and prevented her, 
whilst it occurred, from attending to outside work on. ac- 
count of the distortion of her features that it entailed. 
This “disfigurement,” she explained to me, was a large 
swelling that took place daily between 8 and n a. m., 
which appeared in the upper lip and caused it to rise to 
the level of her nostrils, obstructing the nares and pro- 
jecting over two and three inches beyond the surface of 
the teeth. The swelling came on gradually, but continued 
to increase for some hours until it reached a maximum 
about 12 m. or i p. m., when it gradually subsided, so 
that by three, four or five in the evening it had totally dis- 
appeared, leaving no trace behind it. 
The swelling was perfectly painless, and to use her ex- 
pression was “ doughy ” to the feel, and only bothered 
her by its ungainliness. 
I must state that I was at once much struck by the sin- 
gularity of the phenomenon, and especially by the clear- 
ness and accuracy with which the signs and symptoms were 
detailed to me. The periodicity of the affection and its 
long duration, above all, were curious. The swelling 
“ came on regularly every morning and disappeared every 
evening; sometimes, though rarely, it appeared in the 
evening and disappeared late at night,” was the answer 
always given by the patient when interrogated on this 
point; and other women, relatives and neighbors, who were 
about, all substantiated the statements by their repeated 
reiterations. I asked if any chill accompanied the appear- 
ance of the swelling, if it was followed or associated with 
fever or any other general disturbance, and to these que- 
ries the patient always gave a negative answer, stating, in 
addition, “that she was not prevented from doing any 
work about the house by the presence of the enlargement, 
and it was only by the inconvenience of its appearance that 
she did not venture out of the house while it lasted.” 
I then examined her mouth for subgingival abscess, 3 
periostitis, dental caries, or other mouth lesions which so 
commonly cause peri-oral swellings, but I failed to see any 
lesion that could reasonably account for the swelling of the 
upper lip, though some of the molars were carious. The 
absence of any inflammatory lesion about the lips at the 
time the examination was made would also exonerate the 
teeth from any participation in the trouble, as oedemas of 
dental or gingival origin are not so transitory in character; 
and, furthermore, are not known to oscillate in such rythm- 
ical fluctuations as this had done for so long a time as 
three months. What, then, could cause such a periodic 
and painless oedema, as I at once characterized the condi- 
tion? Could it be an occult renal, cardiac or hepatic dis- 
ease with albuminuria? The case puzzled and interested 
me. Without prescribing I requested the patient to send 
me some of her urine the next day, and after examining 
her heart, which I found perfectly normal, and searching 
for other evidences of renal or hepatic disease, such as 
oedemas in other localities, etc., I concluded that this 
trouble was not associated with disease of any of these or- 
gans, though I relied on the examination of the urine for a 
final conclusion on the question of renal disease, which all 
that time struck me as the most probable morbid state in 
which such an oedema could arise. The next day the 
urine was carefully examined for albumen, casts and 
sugar, and the specific gravity carefully taken, and I dis- 
tinctly remember that I found it normal in every respect. 
There was no anaemia, no evidence of any dyscrasia that 
could be incriminated; nothing, in short, that an attentive 
clinical survey could seize as an efficient factor in the cau- 
sation of the trouble. 
I decided to see the patient myself during the oedematous 
period, and that very morning, before 12 m., I called at the 
patient’s house and was at once convinced of the truth of 
her assertions, by the remarkable alteration in the outlines 
of her face. Her lips, and especially the upper one, are 
thin normally and of a darkish red hue, but now the upper 
one projected from her face like a proboscis. The lip had 4 
indeed assumed elephantine proportions and impressed on 
the features a curious, grotesque appearance. The en- 
larged labium hung over the lower like an overhanging 
tumor, of a pale, waxy, leaden hue, especially on its mu- 
cous portion. On touching the mass it felt, as the patient 
said, “ doughy,” pitting on pressure and feeling cold and 
almost clammy to the touch. The cheeks did not appear 
to participate in the infiltration and neither did the eyelids, 
the swelling appearing to blend gradually with the remain- 
der of the face on a level with the naso-labial furrow. 
I again examined the mouth to reassure myself, and took 
the temperature under the tongue, but the whole examina- 
tion gave only negative results. 
It was now time to prescribe, as the patient anxiously 
awaited for a remedy that would relieve her of her annoy- 
ing condition, or for an opinion that would enlighten her on 
the prognosis of her case, the demand fora diagnosis being 
thus embarrassingly thrust before me. 
If there was a striking feature in this case it was certainly 
the quotidian periodicity of the phenomenon, the rythmical 
recurrence of the oedema; and if there is a valuable clinical 
criterion of malaria—though notan infallible one—it is peri- 
odicity. Now the word malaria implies usually quinine— 
therapeutically, at least at the bedside, though, I must 
state, that the converse also appears to hold as well, if not 
better, with man}^—i. e., that quinine implies malaria, or 
rather that the test of malaria is quinine. And surely no one 
long engaged in clinical work will gainsay that this test is 
often regarded as the crucial reagent for the detection of 
malaria. 
But I will not digress at this moment. I will state simply 
that in the presence of this perplexing case the periodicity 
of the affection suggested malaria, and the malaria quinine; 
and with this suggestion I was at least temporarily armed 
with a diagnosis and therapeutic indication. I therefore 
ordered quinia sulphate, twenty grains, in capsules, to be 
administered in the evening, and ten more early in the 
morning, at least three hours before the usual appearance 
of the oedema. 5 
Two days after, at 2 p. m., I was much surprised to see 
the patient herself at my office, and what was most satis- 
factory was that she bore no evidence on her face of the 
annoying trouble, even to the presence of the heretofore 
hidrophilic labium. The patient was delighted that the 
oedema had not returned ; she said she had been cured and 
had not delayed in coming to my office to apprise me of the 
news. I could hardly believe that the ailment had radically 
disappeared, and told her that it might return, but to guard 
against a further recurrence of the trouble, and not without 
some curiosity to know the ultimate results, I prescribed a 
capsule containing quinia and arsenic as a basis, to be taken 
three times daily. The next day I learned the patient had 
had a relapse, but not so well characterized as formerly. The 
tonic pills were then reinforced by the administration of 
twenty grains of quinine in the evening. After this the 
swelling did not return, and, as I have had occasion to see 
the patient quite recently, I have learned that she has been 
completely free from any return of the trouble since the 
time when I prescribed for her. 
Such a well marked interruption in the course of dis- 
ease, and its complete arrest following immediately upon 
the administration of remedy, could hardly be regarded as 
a coincidence, and in this I am convinced that the cessa- 
tion of the oedema was entirely the result of the therapeu- 
tic action of the quinine. This practical result, together 
with the peculiar features of the case, did not fail to im- 
press it in my memory. In the lapse of time it became grad- 
ually blended with my malarial recollections. 
It was barely passing into the realm of reminiscence, 
however, when the details of the case were again roused to 
vividness by reading abstracts of two interesting communi- 
cations made at the meetings of June 3 and 25, 1887, of the 
Imperial Royal Medical Society of Vienna, on the subjects 
of acute circumscribed oedema, by Dr. Riehl. In the first of 
these meetings the report says (Semaine Medicate*) that 
Mr. Riehl presented two patients afflicted with acute cir- 
*Semaine Medicate. Paris, Nos. 23, 26. 1SS7. 6 
cumscribed oedema of the skin. “ This affection, which 
has been described by Quincke, consists in the sudden ap- 
pearance and without any appreciable cause of an cedema- 
tous tumefaction, which, in the course of two or three hours, 
becomes very pronounced, and disappears completely at 
the end of twenty-four or thirty-six hours. This tumefac- 
tion is localized ordinarily in the dorsal aspect of the hand 
and in the face, in the eyelids, about the mouth, the lips, 
the cheeks, and sometimes even in the pharynx and larynx, 
where it is liable to produce suffocation.” In one of Dr. 
Riehl’s cases the affection has persisted for three years in 
the zygomatic region ; in the other thirteen years in vari- 
ous parts of the body. At the meeting of the same society, 
held June 25th, Riehl presented further details in regard to 
these two patients. As to the last instance, that of the pa- 
tient who became ill with the disease in 1874, he tells us that 
he is a man aged forty-one years, who had always been 
healthy prior to 1874, when, shortly after the death of his 
wife, he was suddenly seized with oedema in the left eye- 
lid, which, appearing suddenly at night, disappeared as 
quickly the next morning. From this time on and at va- 
rious intervals he presented oedematous localizations in 
various regions of the face. At present, the oedematous 
seizures are repeated every eight or ten days, which, out- 
side of the inconvenience resulting from the altered appear- 
ance, cause no trouble. 
In the other case he referred to a man thirty-three years 
of age, who was attacked three years before, eight days 
after his wedding, by an acute oedema of the left cheek. 
Since that time he has these limited oedemas every fifteen 
days. 
In these two cases the general condition of the patient is 
all that could be desired. The urine is normal and there ex- 
ist no hereditary antecedents. But this is not always the 
course of this affection. In a case reported by Quincke 
the appearance of the oedema was always associated with 
a general malaise, somnolence, vomiting, etc. 
Striibing mentions three cases which were characterized 7 
by intense gastric disturbance, and vomiting as often as 
twenty or thirty times daily, constipation, psychical disturb- 
ances, etc., even when the cedematous areas were insigni- 
ficant in size. Hereditary influences appear to play a part 
in this affection, and apropos of this Striibing relates the 
case of an old man of seventy who was affected with acute 
circumscribed oedema, whose son suffered also with the 
paternal affliction. Analogous examples have been report- 
ed by Quincke, Falcone, etc. 
The differential diagnosis, according to Rhiel, is based 
on the suddenness with which the oedema appears and in 
the absence of all inflammatory symptoms; the absence of 
albumen in the urine or any other evidence that would 
indicate a renal or other lesion that might otherwise reason- 
ably account for it. The differential diagnosis between the 
acute circumscribed oedema and myxoedema should pre- 
sent no difficulties to the clinician. 
The etiology of the disease is still obscure. The influence 
of traumatisms has been appealed to to explain the condition, 
and, in fact, Quincke succeeded in producing an acute cir- 
cumscribed oedema in a predisposed subject, by inflicting 
slight blows on the face, etc., but this mechanical causation 
must entirely play a secondary role, acting purely as an ex- 
citing agent in already predisposed subjects. Moral dis- 
turbances have been regarded, and perhaps with some 
foundation, as important predisposing factors. 
As to the actual phenomenon itself—i. e., the oedema—it 
is accounted for by Quincke, for instance, as an angio- 
neurosis. Striibing attributes it to an exaggerated excita- 
bility of the vaso-dilator nerves. Riehl opines that the 
difficulty lies much more in the central nervous mechanism 
and in its peripheral dependencies, and in support of this 
opinion he appeals to the migratory or metastatic character 
of these oedemas, and their association with nervous disturb- 
ances of a peripheral character and vomiting. 
From the preceeding synopsis of Riehl’s paper, which 
embraces the leading points presented by Quincke (in his 8 
article in Monatscheft fiir Practische Dermatologies No. 
5, 1885) it will be seen that, outside of the leading clinical 
features of the affection, nothing very definite is known as 
to its real nature, notwithstanding the fact that three theories 
have been advanced to explain its main and characteristic 
phenomenon, the acute circumscribed (painless) oedema.