ACANTHOSIS NIGRICANS

Under this title Pollitzer³ and Janovsky⁴ reported and described
minutely, in 1890, each a. case of an obscure and a practically unknown
or unrecognized grave malady characterized in the main by more or less
general pigmentation, associated with, especially on certain regions, the
development of verrucous nævus-like growths. It seems, however,
that a case with apparently similar symptomatology had previously

¹ See report of a successful case, with illustration, by G. H. Fox, Jour, Cutan. Dis.,
1893, p. 166.

² Trimble, Med. Record, July 8, 1905; and (second paper) Jour. Cutan. Dis., 1907,
p. 409.

³ Pollitzer, Internat. Atlas Rare Skin Diseases, 1890, plate x (female, aged sixty-
two).

⁴ Janovsky, ibid., plate xi (male, aged forty-two).

522

HYPERTROPHIES

been recorded by Crocker¹ in 1881. Since the report by Pollitzer and
Janovsky other examples of the disease have been noted by various
observers, among whom are Darier,² Hallopeau,³ Morris,⁴ Kuznitzky,⁵
Neumann,⁶ Spietschka,⁷ Boeck,⁸ Roberts,⁹ Syer,¹⁰ and several others,¹¹
so that now the detailed description of more than 50 cases is on record
(C.J. White).

The onset of the malady is slow or rapid. The pigmentation varies
somewhat in intensity in different cases, being a sallow yellowish,
such as observed in some instances of cancerous cachexia, in others, a
bronze tint, and in still others various shades of a darkish or dirty brown.
It is more or less general, but usually more pronounced about the flexures
and other sites of the papillomatous growths. Concomitantly with
pigmentary changes or following it papillary hypertrophy is noted,
which to a great extent goes into distinct verrucous elevations. The
verrucosity is often limited to or most developed on certain parts,
especially the axillary, genitocrural, anal, and abdominal regions. The
neck, face, lips, and mouth are also favorite situations, and to a variable
extent share in the papillomatous development. The skin, in places at

¹ Crocker, “General Bronzing without Constitutional Symptoms,” London Clinical
Soc‘y Trans., 1881, vol. xiv, p. 152 (with histology—male, aged twenty-two), and
second case, Brit. Jour. Derm., 1899, p. 116 (case demonstration—male, aged fifty).

² Darier, “Dystrophie papillaire et pigmentaire,” Bull. Soc. Derm, et Syph., 1893,
p. 421, and Annales, 1893, p. 865 (female, aged thirty-four), and ibid., 1895, p. 97 (male,
aged thirty).

³ Hallopeau, Jeanselme, and Meslay, ibid., 1893, p. 876 (female, aged seventy-two),
and Hallopeau, ibid., 1896, p. 737 (doubtful case).

⁴ Malcolm Morris, London Med. Chirurg. Soc'y Trans., 1894, vol. lxxvii, p. 305
(female, aged thirty-five).

⁵ Kuznitzky, Archiv, 1896, vol. xxxv, p. 3 (with a colored illustration and histologic
cuts—female, aged forty-one).

⁶ Neumann (case demonstration), ibid., 1896, vol. xxxiv, p. 145 (female, aged
seventeen—case demonstration).

⁷ Spietschka, Archiv, 1898, vol. xliv, p. 247 (3 cases—2 females, aged fifteen and
twenty; male, forty-four—with histologic review).

⁸ Boeck, Norsk. Mag. f. Laegev., No. 3,1897—abstract in Jour. Cutan. Dis., 1897,
p. 588 (female, aged fifty-two).

⁹ Roberts, “Melanosis Accompanied by Moderate Acanthosis” (acanthosis nigri-
cans?) (male, aged fifty-eight), Brit. Jour. Derm., 1897, p. 184 (histologic cut).

¹⁰ Dyer, “A Case of Keratosis Nigricans,” New Orleans Med. and Surg. Jour., 1898,
vol. li, p. 201 (male, aged seventeen).

¹¹ Burmeister, Archiv, 1899, vol. xlvii, p. 343 (reports a case—male, aged thirty-
six—and gives a résumé and analysis of 19 reported cases); Couillaud‘s paper, “Dys-
trophie papillaire et pigmentaire; ses relations avec la carcinose abdominale,” These de
Paris, 1896, and Gaz. des Hôpitaux, 1897, p. 413, gives a review of the subject and
literature to date. An abstract review of the cases and papers by Boeck, Couillaud,
Roberts, Kuznitzky, Rasch, Collan, in Jour. Cutan. Dis., 1897, p. 588, and those by
Barsky, Dyer, and Spietschka, in same journal, 1899, p. 97; Rille, Wien. med. Wochen-
schr., 1897, p. 1019, and Gaucher, Medical Week, 1897, p. 411, give good descriptive
accounts; M. Hodara, Monatshefte, 1905, vol. xl, p. 629 (following a breast cancer);
Wild, Brit. Med. Jour., Aug. 28, 1909 (1 case); St. George and Melville, ibid. (1 case,
with detailed review of literature); Janovsky, Mracek's Handbook, vol. iii, p. 97 (with
literature references); Grouven and Fischer, Archiv, 1904, vol. lxx, p. 237 (with litera­
ture references); Bogrow, ibid., 1908, vol. xciv, p. 297 (with literature references);
Pribram, Deutsch. Archiv f. klin. Med., 1909, p. 407; Pollitzer (“Acanthosis Nigricans:
A Symptom of a Disorder of the Abdominal Sympathetic,”) Jour. Amer. Med. Assoc,
Oct. 23, 1909, p. 1369 (review and analysis of published and two unpublished cases);
C. J. White, Jour. Cutan. Dis., April 1912, p. 179 (1 case, girl fourteen, beginning when
aged four; case illustration and histologic cut); Schalek, Jour. Cutan. Dis., 1912,
p. 660 (woman aged fifty-eight, extensive and well marked case; abdominal tumors
of unknown character; no operation and no autopsy, case illustration).

ACANTHOSIS NIGRICANS

523

least, and especially on those parts most affected, often presents accen­
tuation of the natural lines, in some instances amounting to superficial
furrowing, and is hypertrophied. Palmar and plantar keratosis is
commonly present, and partial or complete loss of hair, especially of the
hair of the scalp and eyebrows, and dystrophic nail-changes are quite
frequently noted. In the region of the flexures the warty growths are
often so numerous and crowded that practical coalescence ensues, re­
sulting in the formation of masses of a papillomatous and vegetating
aspect. These, like the skin, vary in intensity of pigmentation from a
grayish yellow to almost black, and are usually seen in all stages of growth
—some minute, others distinctly verrucous. Scaliness is not a usual
feature. Darier has noted also the development of nævi, freckle-like
spots, and seborrheic warts; and in a few instances epitheliomatous
degeneration in some of the lesions has been observed. The lesions on
the mucous membrane, especially the mouth, are not present in every
instance, but more or less involvement is the rule; they consist of furrows,
discrete or crowded papillomatous formations, which may be sessile or
slightly pedunculated, or the mucous surface may be more or less granu­
lar-looking.

The course of the malady is slow or somewhat rapid, with some
exceptions cases ending, after some months or several years, fatally.
A cancerous cachexia is commonly developed, and in the majority of
instances sooner or later carcinomatous disease of one of the abdominal
organs is recognized—usually the stomach, but occasionally it is the
uterus, and in exceptional cases it is more or less general, sometimes
secondarily to cancer of the breast.

Etiology and Pathology.—The cause of the disease is not
definitely known, but the frequent association of carcinoma, usually
affecting the organs of the abdominal cavity, would appear to make it,
in the majority of cases at least, dependent upon the latter malady,¹
Darier, Pollitzer, and others believing that the interference with the
functions of the abdominal sympathetic thus engendered has a causative
relationship with the cutaneous manifestations. Internal malignancy
has not been noted, however, in the cases under the age of nineteen.
It may occur at almost any age but it is rare in childhood.² According
to Burmeister‘s analysis of this point, in 14 cases 1 case was observed
under the age of twenty years; 1 between twenty and thirty; 3 between
thirty and forty; 5 between forty and fifty; 2 between fifty and sixty;
1 between sixty and seventy; and 1 between seventy and eighty. As
to sex, women seem slightly more prone to it, according to Burmeister's
analysis furnishing 60 per cent, of the cases. Couillaud, whose valuable
contribution was prepared under the supervision of Darier, comes to
the following conclusions:³ “The disease is a syndrome dependent upon
abdominal carcinosis and characterized—(I) from a clinical view­point

¹ See Couillaud‘s and Burmeister‘s and Pollitzer‘s papers, loc. cit., on this point.

² C. J. White, (loc. cit.) in describing his own case, aged fourteen, which began
when patient was aged four, mentions that Pospelow and Buri have each noted a
case at the age of two, and Wolf, Hugel and Spietsche (2 cases) beginning at the age
of three.

³ Quoted from abstract in Jour. Cutan. Dis., loc. cit.

524

HYPERTROPHIES

by: (1) a papillary hypertrophy and a cutaneous pigmentation having
an essentially regional character; (2) a papillary hypertrophy of the
mucous membrane; (3) a dystrophy of the hair and nails; (4) absence
of desquamation; (5) existence of a cachexia; (II) from a pathologic
standpoint, by carcinomatous degeneration of the abdominal organs;
(III) histologically, by lesions of hypertrophy and pigmentation in the
rete and corium.” Histologic examinations show that the horny layer
is thickened, the rete cells, more especially the prickle-cells, enlarged,
the corium infiltrated and exhibiting some mast-cells; and both rete and
corium filled to a variable degree with pigment granules, irregularly
distributed or in masses, partly intracellular and partly extracellular.

Prognosis and Treatment—When no carcinomatous disease
is present, the course of the malady is slow and its outcome uncertain;
when, as in most cases, however, there is underlying carcinoma, a fatal
result is but a matter of months or a few years. Treatment, unfortu­
nately, is purely expectant, with scarcely a possibility of influencing
the course of the disease. Boeck thought that in his case life was pro­
longed by the administration of suprarenal extract. C. J. White‘s
case showed some improvement under thyroid medication.

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