Commentary
CLASSIFICATION OF ACANTHOSIS NIGRICANS
HELEN OLLENDORFF CURTH, M.D.
From the Department of Dermatology,
College of Physicians and Surgeons,
Columbia University, New York,
New York
Acanthosis nigricans occurs in many
countries, climates and races and it may
be seen in various forms. It is important
to know to which type of the disease a
case of acanthosis nigricans belongs, in
order to comprehend its significance.
Malignant Type
The most important type is malignant
acanthosis nigricans, or acanthosis nigri-
cans, which always represents a benign
dermatosis in association with a malig-
nant internal adenocarcinoma. This asso-
ciation is causal and not coincidental,
since both processes usually start at the
same time an dtake a parallel course.
Actually, the dermatosis and the cancer
start simultaneously in 6 1 % of patients;
cancer precedes acanthosis nigricans in
22% and acanthosis nigricans precedes
cancer in 17%. In one instance,^ the pa-
tient had the dermatosis for 16 years be-
fore the cancer became manifest.
The tumor usually is a highly malignant
adenocarcinoma of the stomach, esopha-
gus, cystic duct, hepatic ducts, colon,
pancreas, mammary gland, uterus, ova-
ries, testes, lung or thyroid (?).2 The
disease usually is fatal. In only 2 instances
of cancer of the sigmoid colon did the
patients survive. The patients are non-
obese adolescents and adults, but the
disease has also been reported in children.
592
The dermatosis is usually progressive;
it also affects the mucous membranes
with nonpigmented papillary lesions.
The patients may complain of itching.
They may develop keratoma of the palms
and soles. Keratosis of the nipples and
umbilicus may be present but keratoses
may also be present in benign acanthosis
nigricans.
Since the various components of malig-
nant acanthosis nigricans—hyperkera-
tosis, acanthosis and hyperpigmentation
—do not always develop simultaneously,
it may happen that hyperkeratotic papil-
lary growths (sign of LeserTrelat) become
prominent. Therefore, the Leser Trelat
sign usually represents an early stage of
acanthosis nigricans. There are a few in-
stances in which the transition from Leser
Trelat to malignant acanthosis nigricans
was observed.'^''' Moreover, seborrheic
keratoses on the body or extremities have
almost always accompanied characteristic
lesions of acanthosis nigricans. I have
lately seen a Negro patient who showed
only distinct hyperpigmentation, another
component of acanthosis nigricans, over
large areas of the body in addition to
typical cutaneous changes in the axillae
and a gastric carcinoma.
Benign Types
Acanthosis nigricans not associated
with an internal carcinoma occurs in sev-
eral types. Benign acanthosis nigricans, a
genodermatosis, is Irregularly dominantly
inherited. It is present at birth, or begins
in childhood or at puberty. After that
time it either regresses or remains sta-
No. 8 ACANTHOSIS NIGRICANS
tionary. It is not accompanied by endo-
crine disturbances.
In dark-skinned obese individuals of
both sexes pseudo acanthosis nigricans'
may start at any age. The obesity may be
due to endocrine disturbances, overeat-
ing or constitutional factors. The process
is reversible if the overweight can be
considerably reduced. In acromegaly and
gigantism, a reversal of the cutaneous
process is impossible. In the tropics,
pseudo acanthosis nigricans is due to
sweating and maceration.
Lately, a number of genetic conditions
have been found associated with acan-
thosis nigricans. Such conditions are
Bloom's, Down's and Crouzon's syn-
dromes; lipodystrophy, phenylketonuria,
and others now known. Acanthosis
nigricans also occurred in siblings of 3
sisters .with nerve deafness and absent
gastric motility.'' The dermatosis is pres-
ent at birth or begins in childhood. It
sometimes disappears at puberty.
Characteristics
The clinical appearance of the derma-
tosis is the same in all 4 types. The body
folds show hyperpigmented, confluent,
verrucous skin. The nipples and the um-
bilicus may become keratotic. Sometimes
palms and soles become thickened. In
malignant acanthosis nigricans, the mu-
cous membranes may be affected with
nonpigmented verrucous growths.
The histology of all 4 types is the same.
It shows hyperkeratosis, acanthosis and
hyperpigmentation. The sexes are about
equally affected.
Treatment of the malignant type in-
volves earliest recognition and eradica-
tion of the tumor. Treatment of benign
acanthosis nigricans should be deferred
Curth 593
until after puberty; some cases regress
spontaneously. In widespread cases, der-
mabrasion has proved satisfactory. If
pseudo acanthosis nigricans is caused by
obesity, the excessive body weight should
be reduced. If acanthosis nigricans is part
of a syndrome, the other components are
usually more disturbing than the acantho-
sis nigricans. In some syndromes, acan-
thosis nigricans disappears spontaneously.
The significant of the various types is
vastly different. In malignant acanthosis
nigricans, the dermatosis is an important
cancer clue. Benign acanthosis nigricans,
a genodermatosis, can be greatly disfigur-
ing and the patients may require genetic
counseling. Pseudo acanthosis nigricans
points to an overlying disturbance such
as obesity, Cushing's syndrome, gigan-
tism, acromegaly and others. Acanthosis
nigricans as part of a syndrome may help
in the recognition of the overall
syndrome.
References
1. Freund, E., Cas d'acantbosis nigricans; ap-
parition d'un carcinome apres seize annees.
Rev. Fr. Dermatol. Venereol. 7:450, 1931.
2. Curfb, H. C , Hilberg, A. W., and Macbacek,
G. F., Tbe site and bistology of the cancer
associated with malignant acantbosis nigri-
cans. Cancer 15:364, 1962.
3. Roncbese, F., Keratoses, cancer and "tbe
sign of Leser-Trelat." Cancer 18:1003, 1965.
4. Curtb, H. O., Tovell, H. M. M., and Janovski,
N., Malignant acantbosis nigricans associated
witb endometrial adenocarcinoma. Bull.
Sloane Hosp. Women 8:141, 1962.
5. Curtb, H. O., Pseudo acantbosis nigricans.
Ann. Dermatol. Sypbilol. 78:417, 1951.
6. Hirscbowitz, B. I., Groll, A., and Ceballos, R.,
Hereditary nerve deafness in tbree sisters
witb absent gastric mobility, small bowel
diverticulitis and ulceration and progressive
sensory neuropatby. Birtb Defects 8:27,
1972.