KERATOSIS FOLLICULARIS

Synonyms.—Ichthyosis follicularis; Darier‘s disease; Psorospermosis; Psorosper-
mose folliculaire végétante; Acné sébacée cornée; Ichthyosis sebacea cornea (E. Wilson).

This rare disease was first reported by Morrow¹ in 1886, and a few
years later (1889) was described and thoroughly investigated almost sim­
ultaneously by Darier² and Thibault in France, and by J. C. White³ in
our own country. Since then Boeck, Lustgarten, De Amicis, Bowen,⁴
Lieberthal, Mook, Trimble, Omerod and Macleod, and others have
reported cases.

The disease has been frequently noted to appear first upon the head
and face. In the beginning the lesions do not vary much in color from

¹  Morrow, Jour. Cutan. Dis., 1886, p. 257.

² Darier and Thibault (2 cases), These de Paris, 1889; Annales, July, 1889.

³ J. C. White, Jour. Cutan. Dis., 1889, p. 201 (1 case), and 1890, p. 13 (1 case), with,
in each case, a histologic examination by Bowen. Morrow‘s case, published under this
name (Jour. Cutan. Dis., 1886, p. 257), seems histologically different from those now
accepted as representing this malady, and more accords with the conditions found in
keratosis follicularis contagiosa (Brooke).

⁴ Bowen, Jour. Cutan. Dis., 1896, p. 209, reports a case and gives a review of the
subject; literature references are made to the other published cases by Boeck (5), Buzzi
and Miethke, Bulkley and Lustgarten, Schwimmer, De Amicis, Schweninger and Buzzi,
Pawloff (2), Fabry, Mourek, and Jarisch. It is upon this paper and that by White
that the account of the disease here given is largely based.

Since this date additional cases have been recorded by Hallopeau, Annales, 1896, p.
737, and pathologic anatomy of the same by Darier, ibid., p. 742; Savill (case demon­
stration), Brit. Jour. Derm., 1896, p. 229; Bowen (limited to head and hands), Annales,
1898, p. 6; Graham Little (case demonstration), Brit. Jour. Derm., 1901, p. 51 and
(histologic report) p. 98; Ehrmann, abstract in Brit. Jour. Derm., 1902, p. 41; Ormerod
and Macleod, ibid., 1904, p. 32 (with histologic cuts, review, and complete bibliography);
Lieberthal, Jour. Amer. Med. Assoc, July 22,1904 (good effects from x-ray treatment);
Audrey and Dalous, Jour. Mal. Cutan., 1904, vol. xvi,p. 801 (a woman aged forty-four,
and of twelve years’ duration; urine examination showed a diminution of sulphur); Se-
queira, Brit. Jour. Derm., 1905, p. 266 (case demonstration—woman aged forty-six, of
eight years’ duration); Malinowski, Monatshe}te, 1906, vol. xliii, p. 209 (girl aged seven­
teen, began in first year; review with bibliography, and 4 histologic illustrations);
Mook, St. Louis Courier of Med., March, 1906 (good effects from x-ray treatment);
Jamieson, Edinburgh Med. Jour., Jan. 1907, p. 32 (woman aged thirty-two, three and
one-half years’ duration, beginning simultaneously in the left leg and in the sulcus
behind ears; case and histologic illustrations); Constantin and Levrat, Annales, 1907,
P- 337 (case, male, aged twenty-four, existed since early childhood; mother (Audry
and Dalous case) had same disease); Herxheimer, Dermatolog. Zeitschr., 1908, vol. xv,
p. 45 (3 cases; curative effects of thermocauterization); Grover W. Wende, Jour. Cutan.
Dis., 1908, p. 512 (case, male, aged thirty-seven, beginning twenty years previously,
and resulting in multiple epithelioma; with case and histologic illustrations, review,
and references); Pöhlmann, Archiv. Bd., 1909, xcvii, 1, 2, and 3 (5 cases in 3 genera­
tions—father, 3 children and grandchild); Daisy Orleman Robinson, Jour. Cutan.
Dis., 1911, p. 349 (case demonstration), records a case which presented the combined
features of a seborrheal eczema and lesions resembling those of verruca vulgaris;
Trimble, “Observations on Keratosis Follicularis,” Jour. Amer. Med. Assoc, Aug.
24, 1912, p. 604 (with case and histologic illustrations); 5 cases in one family, 3 gener­
ations—mother, three children, and a grandchild; Ritter, Dermatolog. Wochenschr.,
Feb. 10, 1912, liv, p. 165, case cured by x-ray treatment; Mook, Jour. Cutan. Dis.,
1912, p. 723, 4 cases, all males, aged 18, 21, 24, and 45; one patient stated his brother
had same disease, another that his sister had it; all improved under x-ray treatment.

542                                      HYPERTROPHIES

that of the normal skin; they look not unlike those of keratosis pilaris,
and may have, especially later, when more pronounced and increased
in size, the appearance of greasy-looking papules, or dry, firm, brownish
papular elevations, semiglobular in shape, and varying in size from a
small to a large pin-head. They are at first discrete, and sparsely or
thickly set. When closely examined, most of the lesions—those of any
size—are observed to contain in the center a hardened or fatty-looking
mass or plug. The disease extends slowly, and gradually invades other
parts; finally, usually after several years or longer, it becomes more or
less generalized, being, as a rule, most abundant and showing greatest
development about the face, scalp, the chest anteriorly, the loins, geni-
tocrural regions, and the extremities; with frequently keratosis of the
soles, and less frequently the palms also. Bowen has recently reported
a case, however, in which it was limited to the head and hands. On the
scalp there is usually a thick, seborrheic-looking coating, but no special
hair loss. When at all developed or advanced, the lesions are noted to
have grown larger, and in some places may become confluent, and present
an irregular, papillomatous, or nutmeg­grater-like surface, with sometimes
a fissured appearance or even distinct fissuring. They are noted to be
of various sizes, some of them bearing resemblance to keratosis pilaris,
some larger and containing the firm or fatty central concretion, and others
—a smaller number—rounded or flattened, dull red to dark brown in
color, and exhibiting no central opening, bearing a slight resemblance
to lichen planus papules. Others, again, especially in the advanced
stage, are quite hard and horn­like, of dark-gray or dark-brown color,
hemispheric or conic, and projecting well above the surface. In places
if the disease is at all extensive, elevated areas are formed by confluence
of the lesions, presenting uneven surfaces, covered by thick, yellowish
or brownish, flattened, horny concretions. Less frequently are noted
elongated horny masses, from \ to \ of an inch in diameter, and from \ to
\ of an inch in height, of irregular outline, with blunt, truncated apices,
yellowish in color, of dense consistence, and compactly crowded. They
can be removed with difficulty, and then show bases of corresponding
area, considerably elevated above the general surface, and hyperemic
and moist. On some of these areas are found scattered smaller or larger
crateriform openings, distended follicular openings, filled with firm con­
cretions; occasionally some of these underlying openings show ulceration,
and the whole area is the seat of a mucopurulent discharge. In one case
reported (G. W. Wende) the larger lesions developed into epithelioma.
Close inspection shows that the greater part of the lesions are grouped
about the follicular orifices—in other words, that the disease is essen­
tially a follicular one, at least in its beginning; in some places, however,
where the lesions are confluent and form the papillomatous, irregular,
elevated areas, it can be seen that the process has invaded the inter-
fòllicular structures also. The subjective symptoms are variable—
sometimes intense itching; in other cases, no troublesome features.
The general health remains comparatively undisturbed. A rather weak
mental condition has been noted in some cases. The skin is usually
noted to be of an offensive odor—more particularly in extensive cases,

KERATOSIS FOLLICULARIS

543

and in those in which excoriations have been produced by scratching;
the odor is more or less characteristic of decomposing epithelium (White)
or sebaceous matter.

Etiology.—The age at which the affection begins seems to vary
somewhat; in the larger number of cases, however, it occurred before
the sixteenth year, and in several instances in infancy. Of 24 patients,
15 were males and 9 females. The question of heredity and contagious­
ness has been considered; Boeck had 3 cases in one family, White's
patients were father and daughter, Ehrmann had a patient whose father
had the same malady; Trimble had 5 cases in a family in three genera­
tions (mother, 3 children and 1 grandchild), and Pöhlmann had also 5

Fig. 127.—Keratosis follicularis. (The three cuts of this disease are of the same case).

cases in a family, three generations (father, 1 son, 2 daughters, and 1
grandson); with these exceptions, however, no other support for either
of these possibilities is available. Darier believed he had discovered
the cause in peculiar, coccidia-like bodies in the lesions, and hence sug­
gested the name psorospermosis. This view obtained some credence
and was seemingly supported by Wickham's investigations. Both
observers thought them of etiologic significance not only in this disease,
but also in molluscum contagiosum, Paget‘s disease, etc In later
studies, however, by Bowen, Buzzi, Miethke, Piffard, Boeck, and Darier
himself these bodies were demonstrated to be due to cell transformation,
and not psorosperms, as originally believed.

544

HYPERTROPHIES

Pathology.—Darier‘s psorosperm theory of the origin of the
disease having been abandoned, the view advanced by White and Bowen,
and corroborated since by others, that the affection is evidently in all
its phases a keratosis or modified cornification of the epithelial layers
having its seat in the mouths of the pilosebaceous ducts, has been gen­
erally accepted. Bowen, who has had the opportunity of studying both
of White‘s cases, 2 of his own, and also sections from Lustgarten‘s case,
confirms Darier‘s conclusion as to the follicular character of the malady,
although admitting that the process is not confined wholly to them,
but is found also in their neighborhood. Boeck, on the contrary, can­
not agree, from his investigations, that the lesions are in great part
confined to the follicles. Ormerod and Macleod, from a study of their
case and a review of the subject, conclude, that “it is a type of dyskera-
tosis associated with a peculiar cellular degeneration, which may affect

Fig. 128.—Keratosis follicularis.

any portion of the epidermis, and is frequently located at the upper third
of the pilosebaceous follicle or the opening of the sweat ducts.” The
very smallest lesions are histologically scarcely distinguishable from the
papules of keratosis pilaris, although there are even then traces of the
perverted process of cornification which characterizes the disease. The
“corps ronds” and the “grains” described by Darier are interesting fea­
tures. The former, which were thought to be psorosperms, as already
remarked under etiology, are now known to be transformed cells; they are
found especially in the deeper and middle rete layers, and at the base
of the horny or greasy mass, and probably are, as Bowen states, epidermal
cells that are enlarged and swollen, and made up of a nucleus, with
usually a clear or hyaline protoplasm around it. The “grains” are
probably cells from the bottom of the dilated, funnel-shaped openings
below the follicle plugs, which have become cornified without passing
through the keratohyalin stage. They are rounded, and somewhat

Plate XIX.

Keratosis follicularis in a male adult aged forty ; of many years’ duration,
improvement under x-ray treatment.

Some

KERATOSIS FOLLICULARIS

545

polygonal, shrunken bodies, homogeneous, and with feebly differentiated
nucleus.

As to be inferred, the anatomic changes in the disease are essentially
epidermic—a parakeratosis as well as a hyperkeratosis. In addition
to the evidences of keratosis there is at the periphery of the lesions a
marked increase in the pigment in the normal rete cells. The corium
presents but few changes of any significance—some enlargement of the
papillæ at the sides of the lesions, and a moderate round-celled infiltration
about the vessels.

Diagnosis.—In advanced stages the disease can scarcely be con­
founded with other dermatoses. In the earliest beginning the malady
bears resemblance to keratosis pilaris, but its presence on unusual
locations for this latter affection is a point of difference. Some of the
lesions, especially those on the trunk, may also, early in the disease,
suggest lichen planus, but an inspection of the eruption, as a whole,
would prevent error. At certain stages or in certain lesions the soft
central plug will call to mind molluscum contagiosum; but this latter
never has so wide a distribution, and its pearly-looking characters and
the contained mass with the so-called “molluscum bodies” are points
of difference. Moreover, the opening of molluscum contagiosum
lesions is small, that of keratosis follicularis is, when emptied, crateri-
form. It can scarcely be mistaken for pityriasis rubra pilaris or for
ordinary cases of ichthyosis.

Prognosis and Treatment.—The disease is persistent, and
usually slowly progressive, with periods of greater or less activity.
The general health does not seem to be compromised, although toward
advanced life one would expect to find beginning degenerative epithelial
changes; in G. W. Wende‘s exceptional case epitheliomatous develop­
ment seemed to be a consecutive part of the disease process. No cure
has been reported, but much can be done by treatment to render the
disease less disgusting and disfiguring, and possibly to somewhat restrain
its course. Frequent alkaline baths, as given in psoriasis, and the em­
ployment of stimulating and keratolytic applications, such as salicylic
acid, resorcin, and sulphur ointments, are of decided benefit. Pyrogallol
can also be used in ointment form, but only to limited areas at a time.
Lieberthal's, Mook's, and G. W. Wende‘s cases showed improvement
from x-ray treatment, and in a case now under my own care there has
been also considerable change for the better; Ritter claims to have
cured one case. Herxheimer has had marked success with thermo-
cauterization; he has employed both the Paquelin cautery and the
galvanocautery.

Keratosis Follicularis Contagiosa.—Under this name Brooke¹ de­
scribed a rare affection, apparently of contagious nature, occurring in
children, and sporadically in adults, and characterized by an abnormal
cornification. The first change in the affected parts consists of a slight,
but visible, thickening of the horny layer, with an accentuation of the
cutaneous furrows, and a yellowish to yellowish-black discoloration.
Upon these areas are gradually noted several or more black points, later
¹ Brooke, International Atlas, 1892, part vii, plate xxii.
35

546

HYPERTROPHIES

resembling comedo plugs, seated at the follicular outlets, and of which
one or more develop into papular elevations from which project horny,
straight or bent, spike-like formations of variable length. The regions
usually invaded are the neck, trunk, extensor aspects of the extremities,
and, less commonly, the face and flexor surfaces. The hyperkeratosis,
according to Brooke and Unna, is not limited to the follicles alone, but
extends superficially in their neighborhood and also slightly into the
sweat-pores. Unna states that two main groups of changes are to be
noted: (1) simple appearances of retention and (2) formation of horny
plugs at the seat of the follicles. It is a dry, non-fatty affection. The
process consists essentially of a hyperplasia of the epithelial cells. It is
not improbable, as Unna suggests, that the more localized forms of so-
called acne sebacée cornée of the French also represent this malady.
This writer would include Morrow‘s case of keratosis follicularis in the
same category, the histologic examination (Robinson) giving support
to this opinion. The malady responds readily to simple softening and
alkaline applications.

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