RHINOSCLEROMA

Synonyms.—Gleoscleroma (Besnier); Fr., Rhinosclérome.

Definition.—A neoplastic chronic affection, characterized by an
exceedingly hard, tubercular new growth involving the anterior nares
and region of the nose.

Symptoms.—This disease, which was first described by Hebra
and Kaposi in 1870, usually has its starting-point on the mucosa, of
the nose, particularly of the alæ and septum, and extends very gradually
to the cartilages and skin of the nose and surrounding parts. According
to Wolkowitsch,² in at least 90 per cent, of the cases the nose, both skin
and nares, is the seat of the disease. In some cases the posterior part
of the soft palate and the neighboring organs, as the larynx and trachea,
are the starting-point. As the growth enlarges, the shape of the nose is
gradually altered, becoming broader and flatter, and feels rigid and hard
to the touch, resembling ivory. The lumen of the nasal passages becomes
narrowed, and in some cases completely occluded, due to the inner walls
becoming hypertrophied. The growths in the mucous membrane of the
nose, pharynx; and larynx are flattened, and appear puckered and con­
tracted as they cicatrize. The tumor tends to increase gradually in

¹ Hardaway, Amer. Jour. Med. Sci., April, 1886, p. 511.

² Wolkowitsch, Archiv. f. klin. Chirurg., 1888, vol. xxxviii, p. 449 (an exhaustive
paper from every standpoint).

45

706                                    NEW GROWTHS

size, and the nose, including the lips and choanæ, are implicated in the
process, resulting not only in great disfigurement, but interfering with
nasal respiration, and also more or less with the mobility of the lips.

The cutaneous growths are flat, slightly elevated, sharply defined,
isolated or confluent plaques or nodules; they are painful on pressure,
and very hard, though somewhat elastic, to the touch. They are firmly
imbedded in the cutis and can only be moved with it. Their surface
is normal or reddish in color, smooth or wrinkled, and shiny, and is
traversed by blood-vessels and devoid of hair-follicles. It is impossible
to grasp the affected tissue between the fingers, as it is firmly bound down.
The epidermis is dry, and fissures appear occasionally, secreting a sticky
fluid which forms yellowish crusts. The nodules rarely ulcerate.¹ The
disease pursues a chronic course, extending over years, ordinarily ex­
tremely slow, but steadily progressive.

Etiology and Pathology.—It attacks both sexes alike, and is
usually observed between the ages of fifteen and forty. The affection
is met with most frequently in Austria and Russia, and some cases in
Central America and Brazil. It is quite rare in England and Italy.
In our own country 6 cases have been reported, and all occurred in in­
dividuals of foreign birth, natives of Austria or Russia, with the excep­
tion of 2 instances of the disease in native-born Americans, 1 observed
by G. W. Wende,² in a boy aged eleven, and 1 (woman aged forty)
observed by myself.³ Rona⁴ states that the records show 21 cases in
Hungary, 29 in Russia, 27 in Austria, and 23 in middle America.

The direct cause of the disease is believed to be a special bacillus—
bacillus rhinoscleromatis—which primarily Frisch, and later Paltauf,
Cornil and Alvarez, Payne, Marschalko, and others, found in the tissues.
The bacilli are usually non-motile, occur in twos and fours; are capsu-
lated commonly, and bear a strong resemblance to pneumococci. Accord­
ing to Rona, the bacillus cannot always be found. Secchi states that two
forms of organisms are present, one resembling the blastomyces and the
other resembling a bacillus; the latter, he thought, was a degenerative
form of the blastomyces; the Frisch-Paltauf bacillus he considers a harm-

¹ Zeissl, Wien. med. Wochenschr., 1880, p. 621, noted an instance, however, in which
considerable destructive ulceration had ensued; Rona, Archiv, 1899, vol. xlix, p. 265,
also saw a somewhat similar case.

² G. W. Wende, Jour. Cutan. Dis., 1896, p. 90, with 2 cuts. Among other cases
observed in this country, in foreign-born, may be mentioned by: Jackson, in Hungarian
woman, Jour. Cutan. Dis., 1893, p. 382 (with colored plate); Klotz, in German woman,
ibid., 1895, p. 121 (case demonstration); Allen, ibid., 1900, pp. 282 and 379 (2 cases
—demonstration; in one sloughing had occurred—nationality not stated).

³ Stelwagon, Jour. Cutan. Dis., 1913, “Philadelphia Dermatological Society’s
Transactions” (case demonstration).

⁴ The following three recent papers give collectively, from a dermatologic standpoint,
a complete presentation of the subject and the various reported cases and pathologic in­
vestigations, with bibliographic references: Rona, “Ueber Rhinoscleroma,” loc. cit.
describes 16 of the Hungarian cases; Marschalko, “Zur Histologie des Rhinoscleroms,”
Archiv, 1900, vol. liii., p. 163, and vol. liv, p. 235 (exhaustive complete contribution
on the subject from every standpoint—gives his own studies of 2 cases and a brief
résumé of bacteriologic and histologic investigations, etc, of others, with bibliography
and with 2 plates containing 10 histologic illustrations, some colored); Secchi, “Osser-
vazione sulla istologica ed etiologia del Rhinoscleroma,” Gazetta della Cliniche, Aug.
1, 1899, xix, No. 4, 36, brief abs. in Archiv, 1899, vol. xlix, p. 438; Castex, Jour. mal.
cutan., 1892, p. 161 (review paper with references).

RHINOSCLEROMA

707

less parasite. Besnier and Doyon think the rarity of the malady and its
practical limitation to certain countries are antagonistic to the accept­
ance of the parasitic view. A peculiar degenerated cell is also thought
to be more or less histologically characteristic. Mibelli describes two
cells—a colloidal and a dropsical—practically corresponding to those
found by Mickulicz. Marschalko states that these cells are connective-
tissue cells which have undergone degeneration through the action of
the bacillus, which organism, primarily at least, is inclosed within these
cells singly or in groups. The characteristic cell he thinks resembles
the lepra cell. Upon the whole, there is general accord upon the histologic
features, especially the gross findings. The process is viewed as of
granulomatous character, the corium, and especially the papillary layers,
is densely infiltrated with small cells, which Kaposi regards as being
similar to small-celled sarcoma. Epithelial cells are also found, and
some larger dropsical and colloidal round cells already referred to. True
giant-cells, however, are not present. There is noted in places very
dense fibrous tissue. The epidermis shows, as a rule, primarily at least,
but little if any change, although some observers have called attention
to interpapillary epithelial downgrowths.

Diagnosis.—The great rarity of the malady and, in England,
our own country, and some other countries, its practical limitation to
foreign-born subjects of certain nationalities are to be borne in mind
in considering cases which may be clinically suggestive. The char­
acteristic hardness and the absence of softening and ulceration, its tumor-
like involvement of the nose, usually both within and without, its slow
course and rebelliousness to antisyphilitic treatment, serve to distin­
guish it from syphilis. Upon casual inspection it might be mistaken
for keloid or epithelioma. Keloid is usually preceded by known trau-
matism, and, moreover, seldom occurs about the nose; in doubtful
instances a histologic examination would be decisive, as keloid growths
are essentially different. Epithelioma begins, as a rule, later in life,
rarely involves the upper lip, and usually has infiltrated, often ele­
vated, pearly edges, and with a clear tendency to ulcerate. In
rhinophyma, the extreme development of the third stage of acne
rosacea, the softness of the growth and vascular dilatation, often with
nodules and pustules, are distinguishing features.

Prognosis and Treatment.—The malady is usually pro­
gressive, and hence the prognosis is very unfavorable. It is extremely
rebellious to treatment. The neoplasms have invariably recurred after
operation. Owing to stenosis of the nose, mouth, and larynx, respira­
tion is seriously interfered with, and the patient is unable to take nourish­
ment properly. The general health is not affected during the earlier
progress of the malady, and later only indirectly. Removal of the forma­
tion with the knife and curet may be restored to, but a permanent cure
cannot be expected. The galvanocautery can also be employed to lessen
the nasal stenosis and to check temporarily the invading growth. Lang
speaks favorably of the repeated injection into the tumor of a 1 per cent,
solution of salicylic acid or 2 per cent, solution of sodium salicylate, the
drug also being administered in full doses by the mouth three times daily.

7o8

NEW GROWTHS

X-ray treatment¹ seems promising—Ranzi, Freund, Schein, Pollitzer,
and others having seen favorable influence. Vaccine² treatment has
also been credited with some influence.

But first, if you want to come back to this web site again, just add it to your bookmarks or favorites now! Then you'll find it easy!

Also, please consider sharing our helpful website with your online friends.