CLASS V—ATROPHIES

ALBINISMUS

Synonyms.—Albinism; Congenital leukoderma; Congenital leukopathia; Congeni­
tal leukasmus; Congenital achroma: Fr., Albinisme.

Definition.—A congenital absence, either partial or complete,
of the pigment normally present in the skin, hair, and eyes.

Symptoms.—Partial albinismus, sometimes termed leukoderma,
which, as a rule, involves the skin pigment alone or that of skin and hair,
is identical in its features to vitiligo, except it is congenital, and lacks
the increased pigmentation of the bordering skin observed in the latter
affection. One, several, or many areas, and of various size, may be
present, and they may be rounded or irregular in shape. The skin of
                 the areas is milky­white in color, sometimes with a pinkish tinge; the

hairs are generally likewise colorless. The patches are irregularly dis­
tributed, although, exceptionally, they show cutaneous nerve distribu­
tion, of which Lesser¹ cites an example. In rare instances the albinismus
is limited to one or two patches of hair, and in some of these latter cases
the white lock or locks are noted to be situated about alike through several
generations (see Canities). Partial albinismus, as to the integument,
is most frequently seen in negroes (called “pied” or “piebald” negroes).
As a rule, the patches remain the same throughout life, but in occasional
instances the areas extend, and exceptionally, as in 2 cases—negroes—
noted by Simon,² a tendency to pigmentation is shown.

In complete albinismus the skin of the entire body is milky-white,
with usually, however, a pinkish tinge, due to the integumental blood;
the hair is very fine, soft, and white or whitish-yellow in color, although
in an exceptional instance noted by Folker³ it was bright red. The
irides are colorless, pinkish, or light blue, and the pupils, owing to ab­
sence of pigment in the choroid, are red or reddish-pink. This absence
of pigment in the eyes gives rise to photophobia and nystagmus, noticed
in these individuals, and which also leads them to keep the lids partly
closed during the lightest part of the day, and to avoid brilliant light
exposure. The subjects of this complete form are known as albinos
(Ger., Kakerlaken), and they are noted, as a rule, to be of rather feeble
constitution, and many exhibit imperfect mental development, although
to this are many exceptions. There are no structural alterations in
the skin, there being no departure from the normal other than complete
absence of pigment; and its functions are performed in a perfectly
natural manner. The condition is permanent, although Ascherson,

¹ Lesser, Ziemssen‘s Handbook of Skin Diseases, p. 447 (with illustration).

² Simon, “Ueber Albinismus partialis bei Farbigen und Europäern,” Deutsche
Klinik, 1861, pp. 399 and 406. Almost all the numerous cases described in this paper
are, however, examples of acquired leukoderma—vitiligo.

³ Folker, Lancet, 1879, voll. i. p. 795.

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ALBINISMUS                                          609

Phœbus, and Mayer, quoted by Seligsohn,¹ have noted exceptional
instances in which it partly disappeared; in Mayer‘s case the red color of
the iris disappeared from year to year.

Beyond the influences of heredity no cause is known, and a history of
this etiologic factor is not always obtainable. It is rarely direct, from
one generation to another, the parents usually being free. It is seen in
both blacks and whites. It is quite common for two or three of the chil­
dren to be similarly affected; in fact, a single case in the family is rather
exceptional. In the celebrated Cape May (New Jersey) cases reported
by Marcy² the father and mother were full-fledged negroes, and so far as
could be ascertained there had been no similar instance in the family.
The first two children, males, were black, then came two females, both
albinos, one after the other, then another black female child, and the
last and sixth child, a male, another albino; they had all the attributes of
albinism—cream-colored and silky, though woolly, hair, the pink eyes,
and milky skin. In Folker‘s cases (Caucasians), in addition to the albino
girl with red hair, two other children had the white hair and pink eyes
of the pure albino; the others, five in number, showed no evidence of the
condition; the father and mother were free from the deformity. In the
family observed by Sym,³ in which the complexion was dark in the father,
mother, and relatives, and without previous history of the condition,
of seven children four—the first, third, fifth, and seventh—were albinos,
the others resembling their parents in color. In three cases the irides
were bluish. Lesser (loc. cit.) refers to a family of seven children, of
whom six were albinotic, and Pickel⁴ an instance of a family of thirteen,
of whom seven were albinos; and Mayer, where the second and fourth
children were albinos, the first and third normal. It would seem, by
Boyle's⁵ observations, that the condition in some cases may fail of being
absolutely complete. He cites an example seen among the blacks of
Borneo, whose skin was of a dirty-white color, interspersed with large,
freckle-like spots; the “color of the hair could hardly be described,'‘ the
eyes were pale blue, and he was unable to see well until the sun was low.
The parents of the case had the natural complexion, but his brothers
and sisters were albinos, and many of his ancestors were said to have had
the same blemish. According to Burton, quoted by Beigel, in West
Africa there is occasionally observed a condition which might be termed
semi-albinismus, in which the skin, in color, is between the natural hue
of blacks and whites, and he refers to a case (of which he subsequently
saw a number), of a black, with a café-au-lait-colored skin, hair dull yellow,
but short and woolly, and the eyes a “lively brown.“

¹ Seligsohn, “Albinismus,” in Eulenburg's Real-Encyclopaedie, 1880, vol. i, p.
160; and also by Behrend, in vol. xiii, 1897, p. 476.

² Marcy, Amer. Jour. Med. Sci., 1839, p. 517—also a short preliminary report of
the first children, in Amer. Med. Intelligencer, 1837-38, vol. i, p. 225.

³ Sym, “Albinism—A Curious Family History,” Trans. London Ophthalmolog.
Soc'y, 1891, vol. xi, p. 218.

⁴ Pickel, Blumenbach's Med. Bibl., vol. iii, p. 167—quoted by Lesser, Ziemssen's
Handbuch der Hautkrankheiten, vol. xiv, p. 181.

⁵ Boyle, “Adventures Among the Dyaks of Borneo,” London, 1865, p. 96—quoted
by Beigel (albinismus and nigrismus), Virchow's Archiv, 1868, vol. xliii, p. 529; full
translation of Beigel‘s paper in Amer. Jour. Syphilog. and Dermatol., 1870, p. 136.

39

610

ATROPHIES

In addition to the hereditary factor demonstrable in some instances
other influences have been suggested, especially fright or shock during
pregnancy. This does not, however, seem to be based upon a rational
foundation or upon much clinical support, although the mother of
Marcy's cases attributed the first child to the fright produced, while
pregnant, by the falling down of an old white mare while driving; and
in a case reported by Jefferiss,¹ in an only child, with no family history
of the malady, the mother ascribed it to the strong impression made,
in the first months of pregnancy, upon her mind by seeing an albino.
Aubé, quoted by Seligsohn, is inclined to ascribe the condition to the
marriage of blood relations, and believes the facts of its occurrence in
animals are suggestive of this. Its rather rare appearance, however,
would seem to negative such an opinion.

As may readily be inferred, albinismus cannot be lessened or in­
fluenced by treatment—it is, in fact, without remedy.

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