You are on page 1of 2

tion; and the symptoms tend to get worse.

Lederer rib; these include bossing of the skull, kyphoscolio-


proposes that thepersecution syndrome is psycho¬ sis, cerebral calcification, ovarian fibromas, hypo-
logically caused. He distinguishes this syndrome responsiveness to parathormone, and medulloblas-
from the "K. Z.-syndrome" or concentration-camp toma. The familial nature of the syndrome has
syndrome, which in spite of a superficial resem¬ been satisfactorily demonstrated, but varies with
blance, shows a different constellation and fre¬ the number of possible abnormalities considered.
quency of symptoms. The concentration-camp syn¬ For example, the current investigation is confined
drome, as described for example by Eitinger,3 is to skin lesions and discloses a less impressive pat¬
postulated to be the result of organic changes in tern of heredity than previously reported. No asso¬
the brain produced by mechanical and toxic in¬ ciation with is apparent, but the disease seems
sex
juries as well as by starvation and exhaustion. to be uncommon in Negroes.
In classical German psychiatry, neurosis is not Mason and colleagues were concerned with the
an illness but a faulty reaction pattern due to con¬ possibility of diagnosing the condition from routine
stitutional weakness. As such, a neurosis is not histologie slides. They found the results disappoint¬
considered compensable, and this was the advice ing; in the majority of cases it was impossible to
given by court-appointed psychiatrists to the Ger¬ distinguish nevoid from other basal cell carcinomas.
man courts. Nevertheless, it is a fact that the The jaw cysts examined were compatible with a fol-
courts themselves overthrew this rigid attitude by licular (dentigerous) origin. Ameloblastoma, which
ruling that even constitutionally based symptoms has occasionally been reported, was not found in
are compensable, ". if it is evident that persecu¬
. . their material. The authors conclude that the pa¬
tion experiences have made the predisposition thologist should raise the possibility of a nevoid
manifest, and if it is not certain that such symp¬ origin for a basal cell carcinoma and should indi¬
toms would have appeared in any case. ."2 This. . cate the need for further investigation if the lesion
attitude has been extended further by Eliasberg,4 occurs in a young patient, if there is evidence of a
who expresses the opinion that, where there is multiplicity of skin tumors, if the pattern is that
doubt, one should accept claims if they appear war¬ of superficial multicentricity, or if osteoid tissue is
ranted to common sense and can not be rejected present in the tumor.
on valid reasons by medical science. The importance of a correct diagnosis lies in the
1. Archibald, H.C., and Tuddenham, R.D.: Persistent Stress Reaction
fact that the skin lesions and, to a lesser extent
After Combat, Arch Gen Psychiat 12:475-481 (May) 1965. the jaw lesions, are likely to recur throughout life.
2. Lederer, W.: Persecution and Compensation, Arch Gen Psychiat
12:464-474 (May) 1965. Treatment of the skin tumors must consequently
3. Eitinger, L.: Pathology of Concentration Camp Syndrome, Arch Gen
Psychiat 5:371-379 (Oct) 1961.
be early and conservative, lest gross disfiguration
4. Eliasberg, W.G.: Theory and Practice in Psychiatric Evaluation of result. Note has also been made of the poor re¬
Restitution Cases, Israel Ann Psychiat 2:81-92 (April) 1964.
sponse of nevoid tumors to irradiation. The differ¬
entiation of the syndrome is, therefore, of practical
THE NEVOID BASAL CELL well academic
CARCINOMA SYNDROME as as significance.
1. Howell, J.B., and Caro, M.R.: Basal Cell Nevus: Its Relationship
to Multiple Cutaneous Cancers and Associated Anomalies of Development,

The association of basal cell carcinoma, cysts of


the jaw, and abnormalities of the thoracic cage
was first appreciated by Howell and Caro, in 1959,1
Arch Derm 79:67-80 (Jan) 1959.
2. Gorlin, R.J., and Goltz, R.W.: Multiple Nevoid Basal Cell
Epithelioma, Jaw Cysts, and Bifid Rib: A Syndrome, New Eng J Med
262:908-912 (May 5) 1960.
3. Mason, J.K.; Helwig, E.B.; and Graham, J.H.: Pathology of Nevoid
although a syndrome was not delineated until a Basal Cell Carcinoma Syndrome, Arch Path 79:401-408 (April) 1965.
year later.2 Since 1960, the combination has gen-
erally been called the "basal cell nevus syndrome." KARL ERNST VON BAER (1792-1876)\p=m-\
The most recent of many apposite communications COMPARATIVE EMBRYOLOGIST
appears in the April issue of the Archives of Pa-
thology,3 in which the investigators criticize the
term "basal cell nevus." Originally based on a
clinical misapprehension, the term leads to confu-
Baer, doctor of medicine without a practice,
shifted his interest from gross anatomy to
comparative anatomy, but made his greatest con-
sion between tumors composed of basal cells and tribution in embryology. He described the noto-
those composed of nevus cells. The main presenting chord, advanced the hypothesis of the germ layer,
sign of the syndrome is the appearance of multiple recognized the similarity of the early stages of em-
basal cell carcinomas which are nevoid only by bryonic development in related species, and was
virtue of their early appearance. Therefore, the the first to identify the mammalian ovum. He was
terminology, "nevoid basal cell carcinoma syn- born near Piep, Estland (Estonia), was taught
drome," would seem preferable. reading and writing by a governess, mathematics
Mere association of common signs and symptoms and mathematical geography by a private tutor in
\p=m-\inthis case basal cell carcinomas and bifid ribs\p=m-\ languages, and music, general science, and botany
may lead to the introduction of false syndromes. by a part-time general practitioner.1 Baer began
However, for this syndrome, the regularity of find¬ his study of medicine at the recently founded Uni-
ings conclusively indicates a recognizable symptom versity of Dorpat, Estonia, over the wishes of his
complex in which there may be additional changes parents who preferred one of the German schools.
less common than the lesions of the skin, jaw, and Satisfied with the MD degree received at the age of

Downloaded From: http://jama.jamanetwork.com/ by a New York University User on 05/25/2015


22 but not with the clinical training, he turned to second in 1837. The germ-layer theory espoused by
Vienna, only to discover that he possessed a feeble Baer included four layers, which were revised down¬
interest in bedside medicine. Whereupon he literally ward by Remak into the ectoderm, the endoderm,
wandered over the hills to W\l=u"\rzburg,where Dol- and the mesoderm. The skin and nervous system
linger was directing the research and teaching in arose from the ectoderm; the notochord, the lining
anatomy and embryology. The embryology of the of the gastrointestinal tract, and the digestive
chick was Baer's assignment. Within a year he was organs from the endoderm; and the muscular,
invited to Königsberg as prosector in anatomy. skeletal, and excretory systems from the mesoderm/*
However, before accepting this appointment, Baer The first effect of impregnation is the progressive sepa¬
spent the winter in Berlin, still undecided whether ration of the germ (ovum), the yolk and the membrane of
he should practice medicine. With the appointment the yolk, when the germ begins to increase in size. During
two years later as professor extraordinary, his life the first hours the germ separates from the yolk, but ad¬
work in the anatomical sciences was settled, and heres to the yolk membrane. The elevation of the
. .

the need to practice for a living vanished. germ-layer (Pander's kernel of the chick-embryo) follows .

the yolk-membrane, but it does not come completely clear


The ovum in the graafian vesicle of the dog was of it, but takes some of the yolk-substance with it. Soon
recognized by Baer in the laboratory at Königsberg the center of this elevation is separated by a small amount
in 1827. The discovery was anticipated since he was of fluid from the center of the germ. The germ becomes
convinced that the egg was responsible for the em¬ thinner and firmer, and looks more like a sheet.
With the thickening of the germ the process of division
bryo. This assumption was contrary to the belief of into two layers begins: into the upper, thinner, and firmer
Haller, who postulated that the embryo existed in membrane-like layer, and the lower, thicker, more granu-
an amorphous state in the graafian follicle and the lous and more friable layer. The beginning of the
.

structure appeared only after the uterus was separation probably corresponds with the first stage fol¬
. .

reached. Baer's discovery was described in a letter lowing impregnation. It is noticeable at the twelfth hour,
if the germ is carefully dissected under the microscope.
to the Imperial Academy of Sciences of St. Peters¬ For better understanding I divided the development of
burg, to which he had been elected a corresponding the chick embryo into three periods according to the stages
member. It was prepared simultaneously in Latin of circulation. The first period lasts about two days, until
and in German and was published in Leipzig in the primary circulation is fully developed. The second
1827. The communication is an enthusiastic descrip¬ period lasts as long as the circulation through the vessels
of the yolk sac continues; that is three days, until the
tion of the histology and physiology of the embryo, vessels of the urinary sac are developed and begin to share
with mention of the contributions of Cruikshank, in the circulation. The third period lasts until birth or
Prévost, Dumas, Wolff, and his onetime scientific until the pulmonary circulation appears. The fourth pe¬
riod begins with life outside the egg.
associate, Pander. Each had approached the dis¬ In the development of the skeletal tissue, an accumula¬
covery, but no one had made it.2 tion of the dark granules is seen, corresponding to the
Led on more by inquisitiveness than by the hope of seeing axis of the embryo. The granules form a chain, the spine-
the ovules in the ovaries with the naked eye through all chain (chorda dorsalis).
the coverings of the Graafian vesicles, I opened a vesicle,
of which, as I said, I had raised the top with the edge of As these important contributions were nearing
a scalpel—so clearly did I see it distinguished from the completion, Baer was troubled with inadequate
surrounding mucus—and placed it under the microscope. I financial support for his researches and publica¬
was astounded when I saw an ovule, already recognized tions. He had hoped for an academic post in Estonia
from the tubes, so plainly that a blind man could scarcely
or in a prominent German university. A third
deny it. It is truly remarkable and astonishing that a thing choice was an appointment in the Academy of
so persistently and constantly sought and in all compendia
of physiology considered as inextricable, could be put be¬ Petersburg, which he finally accepted in 1834.
fore the eyes with such facility. Ample funds for construction and research were
Conclusions made available, more time was planned for research,
Every animal which springs from the coition of male and and St. Petersburg was near his homeland. How¬
female is developed from an ovum, and none from a ever, his brilliant observations in embryology were
simple, formative liquid. never systematically expanded. On the contrary,
The male semen acts through the membrane of the the study of embryonic development was substan¬
ovum, which is pervious by no foramen, and in the ovum
it acts first on certain innate parts of the ovum. tially abandoned. The remaining years were devoted
All development proceeds from the center to the periph¬ to the study of physical geography and anthropol¬
ery. Therefore the central parts are formed before the ogy, subjects taught by his tutor in Dorpat. Baer
peripheral. traveled, became a recognized Russian geographer,
The same method of development occurs in all verte¬
brate animals, beginning at the spine.
and accepted assignments for the government fisher¬
ies—but his great work was over. He taught and
In this critical communication Baer made refer¬ lived in Russia until his death.
ence to germ-layers, suggested stages of develop¬
ment, and observed the first rudiment of the dorsal 1. von Baer, K.E.: Life and Letters, Braunschweig: F. Vieweg and
Son, 1886.
spine, later called the chorda dorsalis or notochord. 2. von Baer, K.E.: "On the Genesis of the Ovum of Mammals and of
Each of the structures was better defined and iden¬ Man," Leipzig: L. Voss, 1827; trans. by C. D. O'Malley, Isis 47:117-153,
1956.
tified in a two-volume treatise, Embryology of Ani¬ 3. Baer, K.E.: Embryology of Animals, 2 vol, Z. Danilevicius, trans.
mals, the first volume appearing in 1828 and the (excerpt), K\l=o"\nigsberg: G. Borntr\l=a"\ger,1828 and 1837.

Downloaded From: http://jama.jamanetwork.com/ by a New York University User on 05/25/2015

You might also like