LUPUS ERYTHEMATOSUS

Synonyms.—Seborrhœa congestiva (Hebra); Lupus erythematodes; Lupus seba-
ceus; Ulerythema centrifugum (Unna); Fr., Lupus érythémateux; Scrofulide érythé-
mateuse.

Definition.—Lupus erythematosus is a chronic, mildly or moder­
ately inflammatory, small-celled superficial new growth formation, char­
acterized by one, several, or more circumscribed, variously sized, usually
oval or rounded, discrete or confluent, pinkish to dark-red patches,
covered slightly and more or less irregularly with adherent grayish or
yellowish scales, and seated most commonly upon the face, less frequently
upon the scalp also, and very exceptionally upon other parts.

Symptoms.—Two varieties are encountered, the circumscribed
or discoid (lupus erythematosus discoides) and the more or less diffuse,
scattered, or disseminated (lupus erythematosus disseminatus). The
former is the common clinical type, and is usually seen about the nose,
cheeks, and ears, and less frequently the scalp, and when on the last,
generally conjointly with the disease on the face. It may, however,
be limited to the scalp, for a time at least, and very exceptionally it may
exist on this part for some years without appearing elsewhere² In rarer

¹ Pfannenstiel, Hygeia, May and June, 1910, Straudberg, Berlin, klin, Wochenschr.,
1911, No. 4, and Sequeira, Brit. Jour. Derm., 1911, p. 327, have all seen excellent results,
as has also Forchhammer (cited by Sequeira). The procedure, quoting from Sequeira’s
paper, is as follows: The patient is given 45 grains of sodium iodid internally daily,
divided into six doses. Every morning the nasal cavity is thoroughly cleansed by the
nasal douche containing sodium chlorid and boric acid or other mild antiseptic;
after which it is dried, and tampons of sterilized gauze moistened with a 2 per cent,
solution of hydrogen peroxid are inserted; the patient is provided with the solution
and with a pipet, with which he keeps the tampon well moistened; a result is usually
attained with two to three weeks’ treatment. The action results from the free iodin
liberated in the presence of ozone.

² Stowers, Brit. Jour. Derm., 1898, p. 144, exhibited before the Dermatologic
Society of Great Britain and Ireland, a woman with the disease upon the scalp of
eleven years’ duration, without any manifestation on other parts.

48

754

NEW GROWTHS

instances the hands also show the eruption, but, as a rule, in conjunc­
tion with the patches elsewhere. In most cases, however, coming under
observation the face is the sole seat of the disease, and the flush areas
—nose, cheeks, and ear-lobes—are its most usual sites.

There are no constitutional symptoms except in the disseminated
type, to be referred to later, nor are subjective symptoms present to a
troublesome degree; there may be slight burning or itching, but usually
no local discomfort is complained of. In the discoid type—the common
clinical type—the disease begins as one or several rounded, circumscribed,
pin-head­ to pea-sized pinkish or reddish spots, upon which, if undis­
turbed by frequent washing, slight adherent scaliness is observed. They
are somewhat elevated, at times scarcely perceptibly, in others quite
noticeably, and this is most pronounced at the border. They slowly, or
exceptionally somewhat rapidly, increase in area by peripheral growth,
and, after attaining variable size,—a fractional part of an inch to an inch

Fig. 173.—Lupus erythematosus; a not uncommon situation and configuration (courtesy

of Dr. J. A. Fordyce).

or more in diameter,—they are apt to remain stationary; or they may
increase still further and several contiguous areas coalesce, or a disposi­
tion to retrogression may show itself in some patches, and a tendency
to atrophic change centrally. If coalescence ensues, this, with often
the appearance of new patches nearby, covers considerable area. When
at all developed, the clinical picture is quite peculiar and characteristic:
the patches are noted to be sharply defined against the sound skin by
a slightly or pronouncedly elevated border, while the innermost central
part is somewhat depressed and usually atrophic; the glandular ducts
are generally enlarged and patulous, and often more or less plugged with
sebaceous and epithelial débris; and the entire surface is very thinly and
irregularly covered with grayish or grayish-yellow scaliness, although
this is, as a rule, scanty in quantity. In some cases, however, it forms a
coating with projection into the follicular openings. These cases of
marked follicular involvement represent Besnier’s follicular type. There
is some infiltration or thickening, variable as to degree, but generally
it is slight or moderate. The patch is pinkish or reddish in color,
with frequently a violaceous tinge, the color being most noticeable at

LUPUS ERYTHEMATOSUS

755

the border, at the central part often partly hidden or lessened by the
scales.

Not infrequently the disease is observed to present itself as one
or several patches on the nose and neighboring cheeks, and by growth,
and often by the appearance of new spots in the intervening spaces,
gradually fuse together and form a large area with the narrowed part
over the bridge of the nose, and the outer portion stretching and widen­
ing out on each side more or less symmetrically, like, as Hebra expressed
it, the outstretched wings of a butterfly; this distribution and shape have
given rise to the name “bat's­wing disease.” The whole area, with the
elevated outline border, may be of uniform appearance and thickness,
or thinning and atrophy are noted centrally or here and there in points
corresponding to the centers of the several original constituent patches;
the former is more usual. Other small characteristic patches are fre-

Fig. 174.—Lupus erythematosus.

quently to be seen on outlying regions. Cases of the malady are not un­
common in which but a few fairly large areas present, of a markedly in­
filtrated character, with a prominent border, and which are persistent
and show but little progression or retrogression (lupus érythémateux
fixé of Brocq). In some instances or patches retrogressive changes are
not infrequently noted without atrophic tendency, and the skin, if the
patch disappears, is found to be normal. In other patients, and occa­
sionally in one or two patches, there is very distinct atrophy, so that
the surface presents the appearance of a thin, flat, superficial scar, some­
what sieve-like, showing the previously enlarged duct-openings. On the
ears, lobe and tip, and less frequently in the concha, and the outermost
portion of the canal, it is not uncommon to find patches of the disease,
but not, as a rule, so sharply defined; in patches just inside of the concha,
however, the duct-openings are often quite noticeable and plugged up,
and occasionally dark colored, suggesting an aggregation of comedones.

756

NEW GROWTHS

In some cases the patches are observed to be exceedingly superficial,
almost wholly devoid of thickening or infiltration, the duct-openings
not conspicuous, scantily covered with branny scaliness, having, how­
ever, the sharply defined border. In these instances the areas are
suggestive of mild dermatitis seborrhoica, but do not shade off into the
sound skin, as the latter commonly does. They resemble slightly, too,
when the scaliness is extremely trifling, erythematous patches of erythe­
ma multiforme or chilblains. This represents one form of Besnier’s
vascular or erythematous type. In other cases the patches may be some­
what puffy in appearance, quite a lively red, with often a violaceous tone,
and but little tendency to scaliness, without noticeable duct involvement,
and some appreciable dilatation of the cutaneous capillary vessels;
retrogressive changes are usually more decidedly atrophic than in the
usual clinical types—constituting the so-called telangiectatic type.

In lesions on the hands, concerning which valuable papers have been
contributed by Hyde,¹ Klotz,² Ohmann-Dumesnil,³ and others, the form
of the disease is more usually superficial, not very scaly, and the color
is frequently a violaceous red, sometimes rosy red; they are fairly well
defined against the sound skin, although not so clear-cut in this respect
as generally observed in patches on the face. The dorsal surface is the
usual seat, either of the. body of the hand or the ringers, but the palm
and anterior aspects of the fingers may also be affected. Occurring on
fingers, toes, and pinnacle of ear, it sometimes begins as chilblain or a
simulation of it (lupus pernio). The hands, as already stated, are usually
conjointly affected with the face, although it may occur on this region
primarily or even independently. From the literature review furnished
by the gentlemen named, it would seem that the disease on this region,
although relatively rare, is more common in England than elsewhere.

On the scalp the disease is, while not frequent, not uncommon,
and presents some features different or in a more aggravated degree
than observed ordinarily on the face. Although, according to Besnier,
Brocq, Méneau,⁴ and others, the varying characters of the disease, as
regards discoid, disseminated, superficial, and infiltrated types, may
occur upon the scalp, as elsewhere, the somewhat thick discoid form is
that generally observed. There is not so much redness, as a rule, as on
the face, but usually more thickening, with partial and generally complete
hair loss of the affected area, and rather hard, thick, fibrous, depressed
scar-tissue formation; although here, as elsewhere in the disease, there is
no suppurative action, no ulcerative destruction. The atrophic degen­
erative change is generally so marked that it simulates true scar tissue,

¹ Hyde, “Lupus Erythematosus as it Affects the Hands,” Jour. Cutan. Dis., 1884,
p. 321 (4 cases).

² Kiotz, “On the Clinical Diagnosis of Lupus Erythematosus of the Hand and
Foot,” ibid., 1888, pp. 50 and 90 (2 cases).

³ Ohmann-Dumesnil, “Erythematous Lupus of the Hand,” Amer. Jour. Med. Sci.,
Dec, 1888 (1 case). These several papers go into the subject at length, with good sur­
veys of the literature, with references; in the last an analysis of 46 collated cases is given.

⁴ Méneau (“Lupus érythémateux de cuir cheveleu”), Annales, 1896, p. 579, reports
4 cases and reviews the literature, quoting from various authorities; Dubois-Havenith
records a case, Jour. mal. cutan., 1899, p. 239, limited to the scalp; Galloway, Brit.
Jour. Derm., 1897, p. 329 (case demonstration), exhibited a patient in whom blebs
of some size developed on the scalp areas—a case apparently unique in this respect.

Plate XX.

Lupus erythematosus.

LUPUS ERYTHEMATOSUS

757

and is essentially scar-like in character; it is not usually sieve-like, as ob­
served upon the face. It is somewhat depressed below the skin level.
The hair-follicles are permanently destroyed. The patches commonly
begin insidiously, with a slightly or moderately elevated red border, and
with patulous and frequently stuffed duct-openings, but there is not, as
a rule, much scaliness. While ordinarily not presenting here more than
one to several variously sized patches, it may be exceptionally quite
extensive and coalesce, and involve a greater part of the scalp. The
disease rarely occurs primarily upon the hairy region, but usually sec­
ondarily, and commonly associated with patches upon the face or else­
where. According to my own observations, the malady here is always
attended with atrophic or scar-like changes, rarely disappears sponta­
neously, and always leaves permanent traces—hair loss and cicatricial
tissue.

Lupus erythematosus disseminatus, the exanthematic, or dissemi­
nated type of the malady, first described by Kaposi¹ and since observed
by others (Besnier, Hallopeau, Hardaway, Koch, Cavafy, Pernet, and
others) ,² is, as a rule, a much more serious phase of the disease. It may
develop from the ordinary chronic discoid form, but more frequently
acutely (acute lupus erythematosus) and independently. It is charac­
terized by small, usually numerous, pin-head­ to bean-sized spots or
patches, appearing primarily on the face, where it may remain and cover
considerable surface by the slight enlargement of the original plaques,
but commonly by the appearance of others in the clear interspaces.
They are hyperemic, show, for the most part, but little infiltration and
scaliness, and rarely any marked glandular involvement. The center
may be somewhat depressed, and with or without atrophic tendency.
A certain capriciousness is sometimes noted, old spots disappearing and
new ones presenting. Not infrequently the hands exhibit lesions, and
other parts of the body, as the limbs and trunk, also become invaded.
In some instances there is a resemblance to the lesions of erythema
multiforme,³ and in one instance to the early eruptive patches of granu-

¹ Kaposi, Archiv, 1872, p. 36.

²Hallopeau, Wickham’s Paris letter, Brit. Jour. Derm., 1892, p. 123; Hardaway,
Jour. Cutan. Dis., 1889, p. 448, and 1892, p. 268; Koch, Archiv, 1896, vol. xxxvii,
p. 39 (illustrated); Cavafy, Brit. Jour. Derm., 1897, p. 328; Bulkley, Jour. Cutan. Dis.,
1897, p. 178; Brooke, Brit. Jour. Derm., 1895, p. 73; Jamieson, ibid., 1893, p. 115,
records 2 cases of more or less general distribution, but not acute in development;
Pernet, “Le Lupus Erythémateux Aigu d’emblée,” Etude Clinique, Paris, 1908, records
a case of his own of acute development; gives details of 9 similar cases recorded by others
(Kaposi, 3; Boeck, 1; Koch, 1; Judersohn, 2; Short, 1; Heath, 1 (unpublished); Leslie
Roberts, “Acute Lupus Erythematous” (aign d’emblée), Brit. Jour. Derm., 1911, p.
167, reports another acute and fatal case of this type in a woman, aged 21; father died
of tuberculosis of throat, and his six brothers were said to have died of tuberculosis;
review of these acute cases with observations; Morris and Dore, Brit. Jour. Derm.,
1911, p. 187 (case demonstration; special point of interest the polymorphic character
of the lesions on the hands; when first seen some were like lichen planus, some like
psoriasis, and some like erythema multiforme).

³ Crocker, Jour. Cutan. Dis., January, 1894 (“Lupus Erythematosus as an Imitator
of Various Forms of Dermatitis”), also describes cases of the ordinary benign types
of the disease, in which resemblance was shown to several other eruptions, more par­
ticularly papular and nodular erythema and lichen planus; Engman and Mook,
Interstate Med. Jour., April, 1909, have recently reported several cases in association
with other skin diseases.

758

NEW GROWTHS

loma fungoides (Hallopeau),¹ Very exceptionally vesicular and bullous
lesions have been noted, more especially in the central area of the atro-
phic spots; but in a few instances as beginning lesions. An eczematous
aspect with slight crusting has also been observed. In rare cases there
are attacks, sometimes persistent, of an erysipelatoid condition of the
face associated with the eruption, to which Kaposi especially refers, and
denominated by him “erysipelas faciei perstans.” New lesions often
come out in distinct crops, with symptoms of general disturbance and
febrile action. The eruptive phenomena persist, the disease advances
and often retrogresses, and in many of these patients sooner or later
signs of more or less acute tuberculosis supervene, and death results,
probably more than a majority of the recorded cases ending fatally.
In one under my own observation the eruption was quite profuse and more
or less generalized, partaking of the appearance of erythema multiforme
and lupus erythematosus, with atrophic tendencies in some of the spots;
but as regards the course and patient’s general condition was relatively
benign, although persistent. As in most of these cases, there was a
good deal of burning and some itching.

Lupus Erythematosus of the Mucous Membrane.—In lupus ery-
thematosus the disease is almost invariably one of the integument, but
it occasionally is, conjointly with cutaneous lesions,² met with on the
vermilion of the lip, in the mouth, and on the conjunctiva, usually
extending from the skin of the lower eyelid. On the vermilion of the
lip there may be slight thickening, with scaliness, commonly of a branny
or shred-like character, or there may simply be a rounded, abraded-
looking, rather sharply defined area, with minute sieve-like puncta.
The area is closely similar in color to the lip, but may have a violaceous
tinge. Extending into the inside of the lip on to the true mucous mem­
branes, it has the appearance of a superficial abrasion, but, as a rule,
well defined. The color of the patch when within the mouth—and it
has been observed on various parts—is somewhat variable—some bright
red, and sometimes punctate, others a pale red, the latter often with an
atrophic thinning. On the conjunctiva the surface is congested, usually
sharply defined, and there may be slight thickening, but this is more
likely to be on the ciliary border. In a case under my observation, in
which a patch extended from the nose on to the nasal mucous membrane,
there was considerable infiltration of the latter.

¹ Hallopeau, loc. cit., exhibited a patient before the French Dermatologic Society
in whom the eruptive phenomena appeared to those present to represent the beginning
stage of granuloma fungoides—a year later the case was again exhibited, and the lupus
erythematosus character of the manifestation was evident.

² Some cases in which the mucous membrane was involved have been in the dissemi­
nated type of the disease, an example of which (Petrini’s case) is referred to by Leslie
Roberts, Brit. Jour. Derm., 1897, p. 177. See also paper by G. H. Fox (case of lupus
erythematosus of the face and oral cavity), Jour. Cutan. Dis., 1890, p. 24; also case
demonstration by Lustgarten, ibid., 1897, p. 529; Rille, Wien. klin. Wochenschr., 1898,
p. 1164; Hassler (case demonstration), Jour. mal. cutan., Jan., 1900; Bowen, Twentieth
Century Practice, vol. v (“Diseases of the Skin”), p. 698; Dubreuilh, Annales, 1901, p.
231 (on mucous membrane lesions); T. Smith, Brit. Jour. Derm., 1906, p. 59 (on mucous
membrane lesions); Kren, “Ueber Lupus erythematodes der Lippenrotes und der
Schleimhaute,” Arckiv, 1907, vol. lxxxiii, p. 13 (4 cases, with review and partial bibli­
ography).

LUPUS ERYTHEMATOSUS                              759

The course of lupus erythematosus is essentially chronic and per­
sistent. Although in some cases there occur retrogression and disappear­
ance of old patches, and sometimes without trace, there is almost always
a new cropping-out to take their place, and thus the disease is continued.
In the large majority of the cases, however, the patches are persistent
and progressive, but after reaching a variable size remain more or less
stationary indefinitely. In extremely rare instances, primarily or as a
subsequent development, some slight tendency to the appearance of
flattened, lupus-like tubercles is observed, and the condition is suggestive
of both lupus erythematosus and lupus vulgaris. I have met with two
or three such instances in which the acceptance of a mixed type seemed
the only solution, corresponding clinically to the lupus erythema to-
tuberculeux of Besnier and the lupus érythématoide of Leloir.¹

Fig. 175.—Lupus erythematosus—nose and lip.

Fortunately, in lupus erythematosus, the disease areas, however
long continued, do not show any tendency, as sometimes observed in
old cases of lupus vulgaris to malignant (epitheliomatous) change,
although Pringle² has recorded such an instance and also refers to a few
cases recorded by Dyer,³ Stopford Taylor, and Kreibich; and quite re­
cently Dubreuilh and Petges⁴ have added 2 others.

¹ Spitzer (E. Lang’s Clinic) reports (Annales, 1907, p. 189) a case in which there was
an association of the two diseases (histologically demonstrated); and Kyrle also records
(Archiv, 1909, vol. xciv, p. 309, histologic cuts) a case of lupus erythematosus in which
one of the patches showed histologically the typical picture of lupus vulgaris.

² Pringle, Brit. Jour. Derm., 1900, p. 1; Reyn, Nord. Med. Arkiv., 1911, abt. ii, p.
49—abs. in Brit. Jour. Derm., 1912,p. 375, reports an additional instance, on the nose.

³ Dyer, Daniel’s Texas Med. Jour., 1892-93, vol. viii, p. 178.

⁴ Dubreuilh and Petges, “De l’epithélioma consécutif un lupus erythemateux,^,,
Annales, 1909, p. 106 (2 cases, with review and references).

760

NEW GROWTHS

Etiology.—Lupus erythematosus is not common, and is essen­
tially a malady of early and middle adult life. Kaposi¹ has, however,
seen the disease in a child of three years, and Jamieson² in one of eight,
and exceptionally it has been observed to begin late in life. My own
cases have mostly been between the ages of eighteen and forty.³ While
both sexes are its subjects, more than two-thirds are in women; and
in the acute more or less generalized cases almost all women. Local
congestive conditions and flushings from any cause favor its production,
such as seborrhea, dermatitis seborrhoica, acne rosacea, exposure to the
sun, chilblains, and it has appeared after variola, erysipelas, and similar
disorders. The influence of systemic conditions is not known, but I am
convinced that cases always tend to get worse during times when the
general health is below the standard and when active digestive disturb­
ances and nervous excitement or depression occur. That general sys­
temic states have a material influence is shown by Fordyce’s case,⁴ in
which an extensive eruption appeared early in pregnancy and disappeared
toward its termination; and a second case, disappearing during preg­
nancy and appearing after confinement. Galloway and Macleod and
many others rather favor the view of an underlying toxæmia being an
important etiologic factor.

The main question, however, is the possible relationship to tubercu­
losis. In recent years there has been a growing belief that the eruption
is an expression of this disease, and the evidence accumulating and re­
cently set forth, notably by Boeck,⁵ following that already formulated by
Hutchinson, Besnier, Hallopeau,⁶ Darier⁷ and others, has materially
strengthened this view, with which my own clinical observations coincide.
In more than a majority of the cases of the disseminated type reported
tuberculosis or some suggestive pulmonary disease developed, rapidly
leading to death (in our own country by Hardaway, Fox, Bulkley, and
others). In many of the ordinary clinical types variously reported tuber­
culous tendencies in the families of patients, or the presence of scrof­
ulous glands or other signs of this constitutional state have been noted,
and many of the cases eventually succumb to pulmonary disease. In
fact Besnier and Hutchinson have found tuberculosis more frequently
associated with lupus erythematosus than with lupus vulgaris. Sequeira⁸
and Baleau’s study also show a probable tuberculous relation. Fordyce

¹ Kaposi, Diseases of Skin, p. 509; Schamberg records (Jour. Cutan. Dis., 1906, p.
381, with illustration) a case in a child (girl) aged five years, appearing when aged four
years and three months.

² Jamieson, Brit. Jour. Derm., 1893, p. 115.

³ Two cases in a family are such a rarity that it is worthy of mention that Rona,
Archiv, 1901, vol. 1vi, p. 381, had under treatment 2 sisters aged twenty-four and
twenty-eight; and Sequeira, Brit. Jour. Derm., 1903, p. 171, met with 2 cases in sisters,
one aged seven and the other aged ten; and another instance of 2 cases in two sisters
aged respectively twenty-six and twenty-eight.

⁴ Fordyce, Jour. Cutan. Dis., 1896, p. 89.

⁵ Boeck, Brit. Jour. Derm., Sept, 10, 1898; Ibid., Oct., 1898; Archiv, 1898, vol.
xlii, p. 71; and Trans. Fourth Internat. Derm. Cong., Paris, 1900, p. 108.

⁶ Hallopeau, La sémaine med. 1898, vol. xviii, p. 225.

⁷ Darier, “Précis de Dermatologie,” p. 552.

⁸ Sequeira and Baleau, Brit. Jour. Derm., 1902, p. 367 (disseminated variety asso­
ciated with the presence of tuberculous disease in 70 per cent, and discoid variety in
18 per cent.; history of tuberculoses in the family in not less than 80 per cent.).

LUPUS ERYTHEMATOSUS                              761

and Holder¹ have recently reported a few instances of associated tuber­
culosis, and a most admirable judicial presentation of the subject has
been made by Roth,² who collated about 250 cases of lupus erythema-
tosus, and of these, in over 70 per cent, there was evidence, more or less
pronounced, of tuberculosis. His view, in the absence, so far, of bacilli
findings in the lesions, is that possibly the toxin generated was the causa­
tive agent, which accords with French opinion on the subject. The
association of so-called papulonecrotic tuberculides or other tuberculides,
now and then observed, is thought to strengthen this belief.³ It is only
fair to state, however, that many prominent observers, among whom
Duhring, Kaposi, Crocker, Leloir, Jadassohn,⁴ and others fail to sub­
scribe to this view, although Crocker⁵ admits the undoubted frequency
of the disease in those of tuberculous family history.⁶

Pathology.—In addition to the French view of the disease
being due to the tubercle bacilli toxins, probably by their action on
or through the blood-vessels of the part, other theories, as succinctly
stated in Fordyce and Holder’s papers, “have been advanced from time
to time, such as regarding it as an angioneurosis, as cutaneous inflamma­
tion due to local causes, a specific infectious disease due to micro-organ­
isms; a form of skin tuberculosis produced by a species of bacilli supposed
to differ from those found in the lungs and in lupus vulgaris, a neuritic
inflammation of the skin, the result of the growth of the tubercle bacilli

¹ Fordyce and Holder, Med. Record, N. Y., July 14, 1900 (refer also to suggestive
cases by Jackson and Bronson).

² Roth, “Ueber ide Beziehungen des Lupus Erythematosus zu Tuberculose,”
Archiv, 1900, vol. li, pp. 3, 247, and 395, with brief résumé of all cases reported, with
bibliography; Polland, Dermatolog. Zeilschr., 1894, vol. ii, p. 482, recently met with
lupus erythematosus and erythema induratum in the same patient (woman), who had
lost two sisters from lung disease.

³ Wile, Jour. Cutan. Dis., May, 1911, p. 286, records a case of lupus erythematosus
of face associated with what seemed to be a papulonecrotic tuberculide of the forearm—
the histologic findings are not conclusive, but are somewhat suggestive as to relation­
ship; Bunch, “On Necrotic Tuberculides,” Brit. Jour. Derm., 1912, p. 357, in his two
remarkable cases presented, notes that in one there existed also a well-marked lupus
erythematosus, appearing some time after the tuberculide had developed.

⁴ Jadassohn, “Lupus Erythematosus,” Mracek’s Handbook, gives a good critical
review of the whole subject from the opposite standpoint.

⁵ Crocker, Discussion, Brit. Jour. Derm., 1898, p. 375; Bunch, Brit. Jour. Derm.,
1907, p. 411, took the tuberculo-opsonic indices of 10 lupus erythematosus patients
(from Crocker’s service); in 3 he found the opsonic index to tubercle low; the other 7
showing indices well within the margin of health, in several instances approximating
the normal. In the 3 patients with low index there was found, upon subsequent
inquiry, to be a strong history of tubercle in near relatives.

⁶ The investigations by Zieler, Much, Friedländer and others on tubercle toxins and
Much’s organisms may help to clear up the question—a late review of the subject, with
references, will be found in Friedländer’s two papers: “The Ætiology of Lupus Ery-
thematosus and a report of thirteen cases tested by the Moro reaction” Jour. Cutan.
Dis., 1911, p. 417, and “The Value of Much’s Granules and the Antiformin Method
in Determining the Ætiology of the so-called Tuberculides, with especial reference to
Lupus Erythematosus,” Brit. Jour. Derm., 1912, p. 13; Friedländer is rather non-
committal, inclining to believe it to be due to a toxin, “but whether due to a tuberculous
or other toxin is not so clear”; Freshwater, “Ætiology of Lupus Erythematosus,”
Brit. Jour. Derm., 1912, pp. 57 and 99, also gives an exhaustive review of all sides of
the question, with bibliography—with conclusions unfavorable to the tuberculosis
view, and rather supporting the belief of defective or weakened circulation plus some
irritant from without or within among which might be the tuberculous toxin; and
Ravogli, “Considerations on Lupus Erythematosus,” Jour. Cutan. Dis., 1912, p. 4 (in
support of tuberculous origin with bibliography bearing thereon).

702                                       NEW GROWTHS

in the nerve-fibers in analogy with the skin changes caused by nerve
leprosy.” As yet, however, these several theories are purely speculative.

The cutaneous lesions are, judging from theoretic, clinic, and anatomic
aspects, in all probability a result of two forces: a toxic one from within
(tubercle bacilli products?), acting, as Boeck and others suggest, primarily
upon the vasomotor centers of the skin, and, in the second place, on the
parts of the skin in which the vasomotor disturbances are set up—the
latter being superinduced by such local influences as flushing, seborrhea,
dermatitis seborrhoica, erythema or other local conditions¹ Boeck
states that the main anatomicopathologic changes are vasomotor dilata­
tion of vessels, secondary intoxication of the tissue cells, and inflammation,
the whole resulting very often in atrophy.

The histologic studies (Neumann, Vidal, Thin, Crocker, Fordyce
and Holder, Robinson, and others) of the disease do not agree abso­
lutely in the findings, probably due, in a measure, to the stage and char­
acter or activity of the disease process in the patch examined. Nor
do investigators place the same interpretation upon the histologic changes
observed. One of the latest contributions on the subject is that by Rob­
inson,² who reviews the entire subject and gives, in brief, the findings of
others. While clinically the process would suggest an essential involve­
ment of the epidermic tissue, examinations show that the alterations in
the epidermis (hyperkeratosis, etc) are secondary and unimportant, and
that the principal changes are to be found in the corium, especially about
the blood-vessels, and that the primary lesion is focal in character, and
when fully developed, constituting in reality a new growth, reticular in
structure, and connected especially with the lymph-channels; that there is
an associated perivascular infiltration, most marked where the blood-
vessels are most numerous, as around the glandular structures and hori­
zontal blood-vessels; no giant-cells and no polynuclear cells; the ex­
cretory parts of the glandular structures show some invasion of infiltrated
cells; the plugs are, according to Unna, the result of acanthosis, and are
not sebaceous material. There is variable edema of the prickle layer

¹ Warde, Brit. Jour. Derm., 1902, pp. 332 and 380, 1903, p. 161, believes that lupus
erythematosus is not a disease, but merely a stage in the course of many different affec­
tions—the damaged part, when unable to be self-reparative, undergoing destruction
and replacement by fibrous tissue. He calls attention to its frequent association with
chronic atrophic and hypertrophic rhinitis and ozena, suggestive of an allied nature or
relationship; Galloway and Macleod, “Erythema Multiforme and Lupus Erythemato-
sus: Their Relationship to General Toxæmia,” Brit. Jour. Derm., 1903, p. 81, are in­
clined to ascribe the malady to an inderlying toxemia, which sometimes may produce
erythema multiforme; in others, from lack of reparative power, lupus erythematosus;
also another interesting paper by the same writers, ibid., 1908, p. 65, on the same sub­
ject (with case report), as well as bearing upon the question of relationship to tuber-
losis (with references); Ormsby, “Erythema Toxicum Resembling Lupus Erythemato-
sus,” Jour. Cutan. Dis., 1910, p. 477 (case demonstration; face and hands; more or less
persistent; this and similar cases of simple erythema and erythema multiforme
resemble it, and doubtless sometimes lead to it; Hartzell, “Lupus Erythematosus and
Raynaud’s Diseases,” Amer. Jour. Med. Sci., Dec, 1912, p. 793 (cites cases of his own
and other observers in which there were pronounced vasomotor symptoms, and
believes there is an intimate relation between the two diseases—that lupus erythemato-
sus is a toxic erythema).

Concerning the views of various other observers, see paper by Civatte, “ Les opin­
ions d’aujourhin sur la nature du lupus erythémateux," Annales, 1907, p. 263.

² Robinson, Trans. Amer. Derm. Assoc. for 1898 (with some literature references).

LUPUS ERYTHEMATOSUS

763

and of the cutis. Unna lays stress upon a lymph canalization of the
infiltration. Fordyce and Holder¹ found the sebaceous glands affected
with hypersecretion, and later their ducts, as well as the sweat-gland
ducts, as others have observed, are the seat of infiltration, and subse­
quently undergo degenerative changes, to which the punctate or sieve-
like character of the scar is due. These observers are inclined to believe
that capillary obstruction is the primary step in the pathologic process,
although admitting that it may be the effect and not the cause of the con­
nective-tissue change. Schoonheid² states that the progressive inflam­
matory changes noted lead to typical degeneration of the elastic fibers,
and that it is especially this that is an important factor in the resulting
scar-like atrophy.

From his own repeated examinations and from a review of those
of others Robinson concludes that: “Lupus erythematosus is a chronic
inflammatory disease of the cutis with special histologic characters,
as shown by the changes in the blood-vessels—new blood-vessels in the
affected area, lymph-vessels, and lymph-channels, and the new formation
of an adenoid-like tissue,—reticular tissue,—the presence of mononu-
clear and absence of polynuclear cells in the cell infiltration; and these
changes must depend upon the presence of a poison generated in loco.
In other words lupus erythematosus is a local infective process—a granu-
loma.”

Diagnosis.—As a rule, there is no difficulty in the recognition
of this disease, as its features—the sharply circumscribed outline, the
reddish or violaceous color, the elevated border, the tendency to central
depression and atrophy, the plugged-up or patulous gland-ducts, the
adherent grayish or yellowish scales, together with the region attacked
(usually on the nose, cheeks, ears, or other parts of the face), the slow
course, and the age of the patient are quite characteristic. It is not
to be confused with eczema, dermatitis seborrhoica, lupus, and syph­
ilis, and on the scalp with alopecia areata and folliculitis decal-
vans. The itchy nature of eczema, its diffused character and lack of
sharply defined border, and often the presence of vesicles, papules, or
oozing, with no disposition to atrophy, together with its history, distin­
guish it from this disease. Dermatitis seborrhoica may be sharply bor­
dered, but the greasy or oily character of the scales and crusts, and its
usual association with seborrheic condition of the scalp, and absence of
the other features of lupus erythematosus usually suffice. It is not to
be forgotten, however, that lupus erythematosus sometimes develops
from a seborrhea.

Lupus vulgaris in the vast majority of cases begins in early life,
and there are tubercles, usually a tendency to ulceration, and tough
cicatricial formation—features wanting in lupus erythematosus; in rare
instances the non-ulcerative type of the former, in which the tubercles
consist more of a flattened, diffused infiltration, bears some slight re­
semblance, and, as already noted, a mixed type of the two diseases is

¹ Fordyce and Holder, loc. cit. (with 8 histologic cuts).

² Schoonheid (Ehrmann’s laboratory), Archiv, 1900, vol. liv, p. 163 (an elaborate
investigation, with 6 colored cuts).

764

NEW GROWTHS

within the range of possibility, but confusing examples are, nevertheless,
extremely rare. A confluent patch of flattened, slightly scaly, non-
ulcerating, tubercular syphiloderm may exceptionally, if hastily exam­
ined, be confused with it, but the individual tubercles can usually be
made out on close inspection, and there are no patulous or plugged gland-
ducts, and the sharply defined border of lupus erythematosus is wanting.
The acute disseminated type may remind one possibly of a mild psoriasis
of the seborrheic type or an ill-defined erythema multiforme, but the
tendency to atrophy noted in some of the patches, the constitutional
symptoms commonly present, and the absence of the usual distribution
of psoriasis are points of difference; the same features serve to distinguish
it from erythema multiforme. In fact, this type is so exceptional that
it scarcely needs to be considered.

Patches on the hand are also so extremely rare that the question
of differentiation would seldom arise, but in these cases, even if such
patches be obscure, others usually found on its common sites will be of
aid. Lupus erythematosus of the scalp differs from both alopecia
areata and folliculitis decalvans in its slightly or moderately sharply
elevated border, and the often present patulous or stuffed gland-ducts;
moreover, in alopecia areata there are no inflammatory signs, and in
folliculitis decalvans the patch is likely to be irregular or jagged in shape.

Prognosis.—Lupus erythematosus is a chronic disease, slow in
its course, lasting indefinitely, and extremely rebellious to treatment.
Some cases are capricious, after a time spots disappearing, and some­
times without a trace, in others—the majority—with atrophic scar­
ring. Even in such cases, however, new patches usually arise as others
are retrogressing. In most instances the individual areas are, however,
persistent. After some years the affected parts are seen to be the seat
of flourishing areas interspersed with superficial, soft scars or atrophic
thinning. In some instances, unfortunately few in number, after an
uncertain duration the disease disappears. As already elsewhere stated,
tuberculous or suggestive pulmonary disease has been noted to be the
final development in some cases of the malady, and of which it would
seem to be one of the earliest signals. Upon the whole, however, except
in the acute disseminated type, in which the outlook is always grave,
the patients do not seem to suffer in any way from the disease, either
locally or constitutionally, and its principal rôle and existence seem to be
as "a destroyer of good looks," and, unfortunately, its victims are mostly
women.

As to the possibilities of treatment, the areas present can almost
invariably be benefited, sometimes much improved, sometimes com­
pletely cured; but as to freedom from new spots and a disappearance
of the malady, no positive opinion can be vouchsafed without qualifica­
tion. Some cases respond to persistent measures, and in those instances
in which the disease activity has already ceased, permanent cure results;
in others, especially those in which the tendency to new spots is still
present, treatment is often disappointing.

Treatment.—In the management of this disease both constitu­
tional and external remedies are in most instances to be prescribed.

LUPUS ERYTHEMATOSUS

765

While certain drugs internally administered have from time to time
been extolled,¹ the fact remains, I believe, that the best plan of general
medication which is most likely to have an influence is that which con­
siders the patient, instead of his cutaneous malady; in other words, to
be based upon indications in the individual case. Three conditions,
it has seemed to me, may tend to retard favorable effect from local treat­
ment—digestive disturbances and constipation, a general debilitated
state, and nervous worry or other neurasthenic influences. For the
relief of these the ordinary plans or suggestions referred to in the treat­
ment of eczema can be consulted. The bowels should be kept free, and
all foods and work or indulgences which tend to flush the face avoided.
Alcoholic drinks in any form, as well as hot, rich soups, and pleasures
or employment which require great exertion or stooping, and exposure
to sun or wind, are therefore prejudicial. Likewise excessive coffee-
or tea-drinking and the too free use of tobacco. There are several
remedies which in some cases have seemed to be of special influence.
Of these, in strumous subjects and those of enfeebled nutrition cod-liver
oil, in small or moderate dosage, is sometimes of distinct value. Arsenic
has cured some patients, according to Hutchinson and others, and in
sluggish, persistent cases may be worthy of a trial. Iodid of starch,
according to McCall Anderson, proved effectual in some cases and bene­
fited others Phosphorus in moderate doses has been commended by
Bulkley, and ichthyol in 5- to 15-drop doses three times daily by Unna,
Crocker, and others. Both Unna² and Brocq³ speak well of remedies
which tend to reduce cutaneous hyperemia, or modify the conditions
which produce it, the former commending quinin, digitalis, belladonna,
and ergotin, and the latter ichthyol, ammonium carbonate, and sodium
salicylate. Salicin and quinin in large doses have been warmly spoken
of by Crocker,⁴ and the latter remedy is also endorsed by Eddowes,⁵
Payne, and Hartzell.⁶ Iodoform, originally recommended by French
dermatologists (Besnier and others), but later more or less abandoned,
has recently been credited by Whitehouse⁷ with a cure of an obstinate
and extensive case, given in the dosage of 1 grain (c.065) after each meal.
Of the remedies mentioned, my own observations would give the most
value to cod-liver oil, salicin, sodium salicylate, and quinin, but as these
various drugs are usually administered conjointly with external treat­
ment, it is difficult to gauge more than approximately the amount of
influence they exert. The selected drug is prescribed three times daily in
dosage of—salicin, 10 to 20 grains (0.65-1.35); sodium salicylate, 5 to 20
grains (0.35-1.35), and quinin, 5 to 8 grains (0.35-0.55). Pernet believes
that the mortality of the acute general cases might be reduced by early
confinement to bed and the administration of large doses of quinin.

¹J. C. White, “Lupus Erythematosus: its Amenability to Treatment," Jour.
Cutan. Dis., 1898, p. 457, refers to the various remedies, both internally and externally,
prescribed from time to time for this disease.

² Unna, “The Treatment of Lupus Erythematosus,” Jour. Cutan. Dis., 1898, p. 465.

³ Brocq, “Traitement des maladies de la Peau.”

⁴ Crocker (regarding salicin), Brit. Jour. Derm., 1898, p. 8.

⁵ Eddowes, discussion, ibid., 1898, p. 375.

⁶ Hartzell, personal communication.

⁷ Whitehouse, New York Med. Jour., 1899, vol. lxix, p. 159

766

NEW GROWTHS

The essential part of the management of this disease is, however,
the external treatment, and should be prescribed in every case. The
choice of application is guided by the degree of inflammatory action
or hyperemia and the irritability of the skin. Recent years have seen
the gradual abolition of the destructive methods or, rather, their use
has become limited to comparatively few cases. Upon the whole, as
both the personal experience of White and Unna and their reviews of
the subject show, the mildest applications have the widest field of use­
fulness and are to be depended upon in most instances. My experience
is fully in accord with this, although recent observations with the use of
carbon-dioxid snow, if confirmed by larger experience, might change my
opinion to that extent. As a rule, unless extremely sensitive and irri­
table, the parts are to be washed with soap and water nightly before the
remedial application; and if at all sluggish, the tincture of green soap may
be employed. There are two applications which are often valuable in
all types, but especially in the markedly hyperemic cases, and which I
can commend highly—the calamin-zinc-oxid lotion and the lotion of
zinc sulphate and potassium sulphuret (see Acne). The former is mild,
but nevertheless often making a favorable impression; the latter, origi­
nally suggested by Duhring, is moderately stimulating, but astringent
and almost always well borne. With one or the other of these prepara­
tions it is a good plan to begin the treatment of every case, and later they
can be employed in the interim between more active applications. The
lotion is to be thoroughly dabbed on, both night and morning if possible.
Should roughness, increased accumulation of scaliness, or irritability
arise, it can be omitted for a day or two and a simple ointment applied;
for this latter purpose one of cold cream, with 10 to 20 grains (0.65-1.35)
each of precipitated sulphur and salicylic acid to the ounce (32.), can
be used. After a few weeks, once weekly or every ten days, I am in the
habit of applying liquor potassæ, pure or diluted, according to the con­
ditions, permitting to dry on and painting over several coats of collodion;
this remains on for two to three days, after which the patient again
applies the lotion and thus this conjoint plan is continued so long as good
results. This plan is a modification of that prescribed by Unna, who
applies a paint consisting of 1 to 2 parts sapo viridis and 10 parts collodion,
and which can be made more active by the addition of 3 to 5 per cent, of
salicylic acid.

Another valuable application as a preparatory one, or for intermittent
use, is one of Lassar’s paste with ½ to 1 dram (2.-4.) of precipitated sul­
phur to the ounce (32.), and sufficient calamin, about 1 to 3 per cent.,
to give it the skin tinge. This can advantageously be combined with
energetic washings with sapo viridis or its tincture. Indeed, in the milder
cases this latter, used with any plain ointment, is extremely useful.
Exceptionally, in hyperemic cases, this stronger soap is too irritant.
Another valuable mild method is with soap washing and the nightly, and
during the day too if possible, application of mercurial plaster. Unna’s
ichthyol plaster-mull is also beneficial in some cases. In addition to
these various mild applications ointments of sulphur and ichthyol and
the several sulphur lotions mentioned under acne can often be used with

LUPUS ERYTHEMATOSUS

767

varying benefit. The ichthyol collodion application of Unna—ichthyol
1 part, collodion 10 parts—is a compressing application of some value,
but it is dark colored and temporarily disfiguring. Hebra, Jr.,¹ had
good results from cooling applications—alcohol alone or a mixture of
equal parts of alcohol, ether, and spirits of mint; the application is to
be made frequently,—at short intervals,—and the oftener the better.

If, after a time, no improvement has taken place, then more active
measures are to be adopted, and this is more especially advisable if
the patches are persistent, with no indication of spontaneous or capri­
cious changes. Of these measures painting over the diseased area
(G. H. Fox) pure liquid carbolic acid deserves special mention; if there
are more than several patches, not more than two or three should be
painted at the one time, as there is occasionally some pain from the appli­
cation; it should be repeated a few days after the film-like crust produced
by it has fallen off—about a week or ten days after the painting. In
some instances several repetitions are necessary to remove the patch,
and in others again the effect is slight and temporary, or entirely negative,
and exceptionally aggravation results. The painting of a solution of
salicylic acid in collodion, from 20 to 60 grains (1.33-4.) to the ounce (32.),
is also useful in some cases, repeated according to its effect daily or every
second day for several days, and then discontinuing until the film comes
off, and resuming the painting again, and so on so long as the action is
favorable. Resorcin in alcoholic solution or in collodion—the former of
10 to 50 per cent, strength, and the latter 3 to 20 per cent.—is sometimes
beneficial, but the collodion solution must be used weak at first, as it
sometimes acts with unexpected energy. Solution of silver nitrate,
from 10 to 60 grains (0.65-4.) to the ounce (32.), painted on at intervals
of a few days or a week, will also prove serviceable in some instances,
but it has the disadvantage of discoloration.

A method recently introduced by Pusey, Zeisler, and others, and
one of great value is the use of carbon-dioxid snow (q. v.); this is applied
from 20 to 40 or 50 seconds, according to the degree of thickening, with
a moderate degree of pressure. A patch may need one or more repeti­
tions. The snow acts as a caustic, and should not be used over more
than 1 to 2 square inches of contiguous surface at the one treatment,
although small separated patches may be attacked at the one time.

In cases in which the patches have been long stationary and are
sluggish in character, and which have failed to be influenced by the
milder remedies, stronger or cauterizing applications can be resorted
to, the first in selection being carbon-dioxid snow just referred to. A
point to be kept in mind, however, is that these preparations may pro­
duce scarring, and this in a disease in which patches sometimes disappear
without trace or with but slight atrophic thinning. Among the other
valuable caustic applications, and only exceptionally destructive to any
marked degree, are pyrogallol and arsenical applications. Pyrogallol can
be applied in ointment or paint form; in the former, with salicylic acid,
and consisting of 20 to 40 grains (1.35-2.65) of salicylic acid, 30 to 60
grains (2.-4.) of pyrogallol, and 4 drams (16.) each of simple cerate and
¹ Hebra, Wien. med. Wochenschr., 1899, p. 14.

768

NEW GROWTHS

vaselin; it is applied as a plaster, changing twice daily, and continued
until some action is effected, and then one of the milder applications or
a plain salve used. Its disadvantage is that it blackens the surface for
the time. In collodion with salicylic acid, as first suggested by Brooke,
it is much more active, and the weaker proportions should be first em­
ployed; the formula most commonly prescribed (Brocq) consists of 1
part salicylic acid, 3 parts pyrogallol, and 40 parts flexible collodion;
Bukovsky,¹ following Brooke, recommends even a stronger proportion,
with 16 parts salicylic acid, 4 parts pyrogallol, and 40 parts collodion.
Pyrogallol in collodion often acts with great energy and needs close su­
pervision. Arsenical salve paste, as used in lupus vulgaris, may be tried
in limited obstinate patches; it is destructive, however, if used with too
much freedom. A safer plan of using arsenic, which I have employed
with satisfaction in a few sluggish cases, is that suggested by Schütz,
of painting the patches with a weak solution, composed of 1 part Fowler’s
solution to 4 or 5 parts water twice daily for several days until an inflam­
matory reaction is produced, and then applying soothing remedies until
this subsides, and resuming the paintings, and so continue if improve­
ment is noted. Joseph² indorses the careful application to the areas,
from time to time, of equal parts of lactic acid and water, a mild ointment
to be used in the interim.

Schiff's³ observations as to the favorable action of the Röntgen rays
in this disease have been confirmed by others. I have had good results
in several instances. The method does not seem efficacious in all
cases, in some having no influence whatever and in others producing
aggravation. The Finsen-light method is also one that can be
employed in some cases with excellent results.

Neither Röntgen-ray treatment nor the Finsen treatment is as valu­
able, however, in this disease as in lupus vulgaris. A new plan of treat­
ment, which has proved serviceable in some cases, is that by the high-
frequency current, using preferably the flat, hammer-shaped vacuum
electrode, and at a distance of ¹/₈ to ½ inch from the surface; it should
be applied for 3 to 10 minutes—sufficiently long to bring about some
reaction. It is repeated at intervals of 5 to 10 days, the calamin-zinc-
oxid lotion or the compound lotion of zinc sulphate and potassium
sulphuret being applied in the intervals.

The operative methods which have been commended from time to
time are curetting and punctate and linear scarification; and of these
the scarification methods have been almost entirely abandoned; and
curetting also, whether wisely or unwisely, is not much in vogue at
present. The latter is, however, still a favorite method with G. H.
Fox.⁴ Both linear scarification and punctate scarification are proce-

¹ Bukovsky, Wien. med. Wochenschr., 1899, pp. 1450 and 1500 (a review of the
various plans favored by different authorities).

² M. Joseph, Lehrbuch der Hautkrankheiten, 1898, p. 235.

³ Schiff, “Fortschritte der Gebiette der Röntgenstrahlen," vol. ii, No. 4; abs. ref.
in Monatshefte, 1899, vol. xxix, p. 340.

⁴ In a recent friendly letter of criticism Dr. George Henry Fox, the well-known distin­
guished and skilful dermatologist, protested against the few words I had given to the cu­
retting method in the treatment of lupus erythematosus, and he stated it had in his hands
always been, and is still, the most valuable method of all in the treatment of this disease.

SYPHILIS

769

dures that, as a rule, require several repetitions, usually at intervals of
one or two weeks. In addition to these methods, galvanocauterization
by means of variously shaped cautery-points or knives and by the
Paquelin thermocautery has been in favor with some French derma­­
tologists, employed as in lupus vulgaris. All these operative procedures
are now, however, but little employed, and, indeed, the tendency is to
depend more and more upon the milder measures, and in this respect
the experience of Duhring, White, Crocker, Hyde, Brocq, and most
other observers is fairly in accord.

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