KERATOSIS PALMARIS ET PLANTARIS

Synonyms.—Tylosis palmæ et plantæ; Ichthyosis palmaris et plantaris; Keratoma;
Keratoma palmare et plantare hæreditarium; Symmetric keratodermia; Fr., Kéra-
todermie plantaire et palmaire.

Definition.—Hypertrophy of the corneous layer of the palms
and soles, usually of a more or less horny and plate-like character.

Symptoms.—The features of this somewhat uncommon malady,
to which the contributions of Unna¹ and Hyde² first directed special
attention, are in their essential character analogous to those of cal-
lositas, but the hardening and thickening arise spontaneously with­
out necessarily having any external factor, such as pressure, friction,
etc, as in the latter malady, and is, furthermore, symmetric, and usually
on palms and soles. Moreover, it is, as a rule, congenital or a hereditary
condition. The degree of development varies somewhat both as to
thickness and uniformity. In the typical cases the whole palmar and
plantar regions are the seat of a thickened, usually smooth, hardened,
and sometimes seemingly translucent, yellowish, brownish-yellow, or
yellowish-gray calloused epidermic plate. It is of a hard, leathery con­
sistence, not infrequently being almost horny in character. While
it is commonly limited to the palmar and plantar aspects, occasionally
it extends somewhat beyond on to the side, and exceptionally slightly
on to the dorsal surface. Much more frequently, however, the only
parts calloused over, in addition to the usual sites, are several or more of
the knuckles. It is common to find the nails more or less affected, and
tilted slightly or moderately upward by the collection of hardened and

¹ Unna, “Ueber das Keratoma palmare et plantare hereditarium,” Archiv, 1883, p.
231 (2 cases, with illustration).

² Hyde, “Observations in Three Cases of Symmetrical Hand and Foot Disease,”
Med. News, 1887, vol. li, p. 416 (3 cases, bibliography). The subject is well gone over
in the papers and discussion in Trans, of Third Internat. Dermatolog. Congress, Lon­
don, 1896. The clinical appearances of the malady are well shown in the plate in
Mracek‘s Hand-Atlas of Skin Diseases, and also in the colored illustration in Crocker‘s
paper, “Tylosis Palmæ et Plantæ,” in Brit. Jour. Derm., 1891, p. 169.

KERATOSJS PALMAR IS ET PLANTARIS                  529

thickened epidermis under their free borders. At the edge of the plate-
like thickening there is generally a narrow pinkish or reddish halo or
zone, apparently passive in character, and not due, as a rule, to inflam­
mation, this latter, with few anomalous exceptions, being observed only
as an occasional accidental factor. In some instances there is associated
hyperidrosis of the parts, in which event the epidermic mass is not so
hard or horny, and although still tough, may have a slightly sodden
character.

The condition is a persistent one, although in some cases, from
time to time, there is a partial or almost complete casting off of the
hardened plate, and a variable intermission of at least comparative
freedom. While the surface is usually smooth or not conspicuously

Fig. 125.—From a case of symmetric keratosis of palms and soles (has occurred in

three generations).

rough, sometimes it is somewhat irregular, and in occasional instances
it has a slightly worm-eaten appearance. The thickness of the plate
varies, averaging almost ¹/₈ of an inch, and sometimes much more over
parts subjected to pressure. The conditions are practically the
same on both palms and soles, although on the latter the hollow of the
foot usually escapes completely or is but relatively slightly affected.
There are variations, however, from the malady as described, and it
may not involve the entire parts uniformly. Brocq¹ has described a case
in which the calloused formation was of a more or less band-like char­
acter and of a longitudinal direction, running along and corresponding
to the middle of the anterior aspect of the fingers. In other instances
the chief thickening is over the joint prominences. In some, moreover,

¹ Brocq, Traitement des Maladies de la Peau, second ed., p. 378.
34

530                                      HYPERTROPHIES

the keratosis consists, primarily at least, of small rounded or conic cal­
losities, with but slight or moderate thickening of the intervening skin.
A case somewhat similar to the last, with a slight erythematous or in­
flammatory halo or zone surrounding the callosities, has been noted by
Besnier.¹ Brooke² has also called attention to a peculiar erythematous
condition (erythema keratodes of palms and soles), seemingly allied to
the malady under consideration, but in which there was some under­
lying erythema, giving the slightly thickened epidermis an orange-gray
color and a quite pronounced inflammatory halo; the inflammation was
of a mild or moderate grade, although the outer edges were somewhat
swollen and tense and hot to the touch. There was, in addition, ery-
thematous horny papules over the joints on the dorsal surface of the
fingers. The malady was less marked on the soles. It responded to
treatment, but was prone to recur.

There are no subjective symptoms—occasionally slight tenderness
around the edges and over the joints. There is interference with free
mobility of the parts, and sometimes fissures are to be seen, and these
are usually quite painful. Exceptionally, from accidental irritation or
as a result of occupation, an eczematous element may be superadded.
Ordinarily, however, the condition, beyond its unsightliness and incon­
venience, gives rise to no trouble. Hyde³ mentions that in these patients
“the pulse is sometimes exceedingly slow, running in adults from 50
to 55 beats a minute, without other manifest impairment of the general
health.”

Etiology and Pathology.—Beyond the fact of the malady
being congenital and often hereditary, but little is known as to its
causes. Exceptionally it has been acquired. A history of its occur­
rence in two or more generations is sometimes obtainable (Thost, Unna,
Date, Crocker, Sherwell, Heuss, Neumann, Pendred),⁴ and not infre­
quently two or more members of the same generation, as in several re­
ported by those just named. It has also been stated that it tends
to affect only one sex in the family, but this is not borne out by an analy­
sis of the cases. In some instances pressure and friction have seemed
to be exciting, or at all events aggravating, causes. The malady is not,

¹ Besnier, “Keratodermia symmetrica erythematosa,” Internat. Atlas Rare Skin
Diseases, 1889, plate v.

² Brooke, Brit. Jour. Derm., 1891, p. 335, with colored plate; also “Notes on Some
Keratoses of the Palms and Soles,” ibid., p. 19; Dubreuilh, ibid., 1892, p. 185, reports a
somewhat similar case.

³ Hyde, Morrow‘s System, vol. iii (Dermatology), p. 405.

⁴ Thost, Ueber erbliche Icythyosis palmaris et plantaris, Heidelberg, 1880, quoted by
Unna (4 generations); Unna, loc. cit. (1 case, 2 and 1 case, 3 generations); Date, “He­
reditary Ichthyosis,” Brit. Med. Jour., 1887, ii, p. 718 (5 generations; brief note);
Crocker, loc. cit. (one instance 5, and another 2, generations); Sherwell, Jour. Cutan.
Dis., 1898, p. 451 (case demonstration—2 generations); Heuss, “Keratoma palmare et
plantare hereditarium,” Monatshefte, 1896, vol. xxii, p. 405 (3 generations); Neumann,
“Ueber Keratoma hereditarium,” Archiv, 1898, vol. xlii, p. 163 (7 plates, 2 generations);
Pendred, Brit. Med. Jour., 1898. i, p. 1132 (3 members of family; disease appeared
in unbroken succession—5 generations—for at least one hundred and fifty years in the
same family, principally through the female line); Vörner (Archiv, 1901, vol. lv, p. 1,
with bibliography), Pasini (Giorn. ital., 1902, vol. xxxvii, p. 318, with bibliography),
Decroo (Jour. d. sci. med. de Lille, July 4, 1903—abs. in Brit. Jour. Derm., 1903, p.
377), and Böhn (Dermatolog. Centralbl., 1904, March, p. 162) also report instances of
the malady through several generations.

KERATOSIS PALMARIS ET PLANTAR IS                  531

however, to be confounded with the keratosis, often of similar general
aspect, such as the callosities resulting from occupation and that noted
in connection with eczema, pityriasis rubra pilaris, and other chronic
diseases; nor that due to the continued ingestion of arsenic (see dermatitis
medicamentosa), although this latter might readily be considered as an
acquired or accidental example of the same malady due to a definite
etiologic factor. It is not improbable that there are several distinct
varieties, as indicated by the unusual types referred to, and that the
etiologic factors are somewhat varied. Besnier divided the cases into
four classes: (1) The ordinary symmetric congenital and hereditary form;
(2) the symmetric keratodermia developing in childhood, of an erythem-
atous and irritable character, and probably connected with some neu­
rosis; (3) symmetric keratodermia, especially of the feet, developing
primarily in isolate foci, and probably of central origin; (4) accidental
keratodermias, distinct from ordinary callositas, occurring at any age,
and provoked by some unusual occupation or work.

While the malady may be seen in any station of life, it is usually
observed in the poorer and working­ classes. It is met with in both
sexes. The condition, or an analogous affection, seems to be endemic
on the island of Meleda, off the coast of Dalmatia, and is known as
the “mal de Meleda”; Hovorka,¹ viewing it originally as a form of
leprosy, subsequently (Hovorka and Ehlers)² retracted this opinion.
Professor Neumann, who visited the island and examined several cases
(loc. cit.)y also dissented from this view. He found that it was not
leprosy, but a disease similarly or closely allied to symmetric keratoder-
mia. In the cases there, however, the thickening was not limited to the
hands and feet alone, but the lower leg and lower forearms and the patellar
region also were involved. Neumann believes it belongs to the category
of the hereditary anomalies of the skin.

Pathologically, the disease is closely related to callositas, and about
the same histologic characters are disclosed, the chief and constant
factor being the thickening and hardening of the corneous layer.

Diagnosis.—The symmetric character of the disease, its usual
involvement of all extremities, the absence of inflammatory symptoms,
the frequent association of hyperidrosis of the parts, and the common
history of its existence since birth and of hereditary tendency will gen­
erally serve to prevent its confusion with thickened squamous eczema,
ordinary callosities, and the thickening occasionally seen in connection
with other maladies. The possibility of a similar or closely similar
condition being due to the prolonged ingestion of arsenic is not to be
overlooked, nor that such keratosis, after once thoroughly established,
is sometimes persistent.

Prognosis and Treatment.—The condition is irremediable as
to permanent relief, but treatment can do much to keep the malady in
abeyance. But little, if anything, is to be expected from general treat­
ment, although Brocq advises large doses of sodium arseniate; and in

¹ Hovorka, “Ueber einen bisher unbekannten endemischen Lepraherd in Dalma-
tien,” Archiv, 1896, vol. xxxiv, p. 51.

² Hovorka and Ehlers, “Mal de Meleda,” Archiv, 1897, vol. xl, p. 251.

532                                  HYPERTROPHIES

the symmetric erythematous keratodermia the same drug, together with
the bromids, and the application of revulsives to the nape of the neck.
Brooke thought the internal administration of ichthyol, 3-minim doses
(0.2), in association with local treatment, of curative value. Klotz¹
believed in one case benefit derived from the internal use of pilocarpin.
The important and usually only treatment which has any effect consists
in external applications, and the most valuable of all are those in which
salicylic acid is the active constituent, the treatment being the same,
in fact, as advised in ordinary callositas (q. v.). A strong salicylic acid
plaster seems, in my experience, the best method of its application—15
to 25 per cent, strength. A 10 to 20 per cent, salicylated soap-plaster,
as advised by Klotz, is also valuable. Soft-soap (sapo viridis) cataplasms
and hot-water soakings may also be used to soften and remove the
hardened accumulation; or instead of sapo viridis, caustic potash solu­
tion, 10 to 30 per cent, strength, can be cautiously employed. In a few
instances frequently repeated short exposures to the Röntgen rays have
been followed by a disappearance of the thickening.

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