XERODERMA PIGHENTOSM, 
BY BUCHANAN KLOPHEL, M, D,, 
MEIEFEZIS, TE1T1T. 
Reprint from the N. Y. Medical Record, June 2d, 1888. DR. R. I TAILOR'S CASES OF XERODERMA PIGMENTOSUM. Xeroderma Pigmentosum. 
Sir :-The very interesting and instructive paper by Dr. 
R. W. Taylor, on " Xeroderma Pigmentosum,1' in The. 
Medical Record of March 10th, was read by myself with 
more than ordinary interest, and, considering the chaotic 
state of every department connected with that affection, 
I take it that, in giving my views on the whole subject, I 
shall be guilty, possibly of no greater sin than a breach of 
modesty. As we are all earnest laborers in the interest of 
mankind and naught but good can come from an inter- 
change of views, though the humblest contribute. But to 
our subject. I was not a little disappointed to find little 
or no mention of the pathogenesis of the disease, and as 
to treatment, to find the most intelligent efforts fruitless. 
Hence if my ideas of its genesis, pathology and treatment 
are far from the truth and ill-founded, we are none the 
worse off. I have never met with a case of xeroderma 
pigmentosum that I am aware of. However, in this con- 
nection it might be of interest to relate the following : Two 
years ago I was treating a young lady for acne rosaca. 
There existed and persisted beneath the left eye a small 
red spot after the acne was cured. This spot I took to be 
a naevus. There subsequently appeared another in the 4 
vicinity of the first. Neither became pigmented; the epi- 
dermis was not raised. I concluded to treat them both 
by igni-puncture, puncturing the periphery thoroughly. 
The spot which appeared first was no larger than a pea, 
while the second was just visible. The effect was satis- 
factory ; there was no further development in them or 
their vicinity, and they disapp eared with scarcely any 
trace. It is questionable if this were a case of incipient 
xeroderma pigmentosum, yet, had I allowed it to develop 
unmolested, it might have proved itself to be such. E'unk 
it is stated in the paper, believes the disease to be one of 
microbic origin. I am also firmly of that opinion, after a 
comparative study of the two possible etiological factors, 
viz., some disarrangement in the order of the epiblast or 
mesoblast, or both, and the microbic. 
To my mind the weight of evidence is in favor of the 
microbic theory; e.g., \Hre it of embf^Jnal origin we 
would, beyond all question, have met the disease more 
frequently. Instead of a few cases and reporters, up to 
date, they would have been legion. Further, it would seem 
that all the cases so far reported had occured in largi 
cities. Although the social plane of none of the sufferers 
is given, I presume it was poor. Ordinarily the disease 
begins with an erythematous patch, or a number of them, 
beneath one of the eyes. It is curious why it should se- 
lect this particular part of the facial epidermis. Obvious- 
ly if it were due to errors in the arrangement of either 
the mesoblast or epiblast, or both, we would not expect 
it to occur in any pirticular spot; though as the order of 5 
the embryonal layers in forming the orbit is complicated , 
and cysts and embryonal tumors are common to that 
region, these are governed by laws, developing at puber- 
ty, or in old age, or following traumatism, etc. Malignant 
affections of the skin, of embryonal origin, occuring 
among children are remarkable for their rarity; while 
serious, not to say malignant, diseases of that tissue, which 
are of parasitic origin, are exceedingly common. I have 
assumed that the morbific agent in the causation of xero- 
derm<^pigmentosum is a parasite, and that the xerodema 
is the result of the direct action of the parasite upon the 
embryonal cells of the deep layer of the rete mucosum, 
and that the pigmentation is the sequel, likewise the car- 
cinomata. It seems to me that the parasite elects to at- 
tack the epidermis beneath the eyes for three reasons : 
i, The skin in that locality is delicate or less corneous 
I think, than other parts; the epidermis about the eyes 
of children, as all know, is more frequently irritated and 
subject to abrasions; 3, the epiderma here, in children, 
is almost constantly bathed with tears. With these prop- 
o|sitions I would connect the following : 
It is noticeable that in all cases, and those of the au- 
thor of the paper in particular, the disease was more ma- 
lignant in the upper part of the face above the mouth, 
and it also spread with greater rapidity in that region, 
and as it extended, or rather the farther it got from that 
locality, the less malignant it appeared. As remarked 
before, the disease starts with a well-marked, smooth, 
superficial erythema. This, it seems to me, would point 6 
clearly to some interference, with the physiological pro- 
cesses taking place in the rete mucosum and corium, in 
fact, a pathological process in loco, viz., a destruction of 
the protoplasm of the cells of the rete mucosum-in other 
words, a thining of the epidermis and an elongation of 
the capillaries of the papillae. In my opinion the degree 
of redness depends upon the above conditions in the 
rete and corium. I may state, also, that I believe that the 
essential pathological changes are the work of a specific 
pathogenic micro-organism, which flourishes in the nitro- 
genous protoplasm of the young cells of the rete mucosum. 
In regard to the relationship between the red and pig- 
mented phases, in the progress of the disease, I cordially 
agree with Dr. Taylor. It is more than likely, "with re- 
gard to the production of pigment," there first takes place 
an elongation of the capillaries, " following the destruc- 
tion of the rete above and between them," with dilation- 
in fact, the production of telangiectasis. Pick says the en- 
dothelium is swollen and folded so that it obstructs the 
lumen. As a matter of fact, a capillary hemorrhage 
would follow the degeneration of their epithelial walls. 
Hence I would account for the pigmentation upon the fol 
lowing hypothesis : The chemical and physical decompo- 
sition of the haemoglobin by light, heat, and possibly the 
presence of an acid--"The product of organic decompo- 
sition, as in all probability varieties of non-pathogenic or- 
ganisms are associated with those of the disease," with the 
production of haematin. Ziegler, in writing of cutaneous 
extravasation (" Pathological Anatomy," sect, iii., pp. 
100, ioi), says : "A third portion of the corpuscles shrink 
up or crumble down into brown granular masses." This 
lies free in the tissues, and often becomes darker in color 
with age" (Ibid). Gusinbauer has referred the produc- 
tion of melanin in pigmented tumors to a decomposition 
of the blood escaped from thrombosed vessels within the 7 
tumor. Ziegler, farther on, in reference to cutaneous ex- 
travasations, remarks as follows : "A fourth, and that 
the greater portion of the corpuscles, and the products of 
their disintegration, is taken up by leucocytes, which 
gather in large numbers round the seat of extravasation, 
and even penetrate into it. The cells become, in this 
way, corpuscle and pigment carriers. The transforma- 
tion of the hematin into brown pigment is completed 
within these cells, the corpuscles breaking up into minute 
granules The pigment may be set free again by the dis 
integration of the carrier cells. Probably the cells have 
also the power of eliminating it without themselves perish- 
ing." 
Dr. Taylor quotes Neisser on this subject as follows : 
"The pigment is distributed in isolated spots similar to 
melanotic tumors, and is seated in the deep layers of the 
epidermis, and rarely m the corium. The pigment is 
precisely similar to that of melanotic new growths, and 
differs from that of ecchymosis ?" Pick, in reference to 
this matter (Ibid.), says : "Pigment deposit keeps pace 
with the invasion of the vessels, and with the small-cell in- 
filtration of the corium. The cell infiltration is undoubtedly 
the carrier of the pigment.'1'1 
As regards the develop|ment of the keratosic patches, 
there is plain evidence of increased nutrition. On the 
other hand, in atrophy we have sufficient evidence of 
nutritive changes, and their cause, i. e., a disappearance 
of the capillaries and their essential environments. It is 
well to note in this connection that the disappearance of 
the capillaries is coincident with that of the pigment. 
Hence a priori, the capillaries have much to do indi- 
rectly with every phase of the disease. 
With reference to the developfment of carcinoma from 
the innocent-looking papilloma, or the keratosic patch, I 
would say that Cohnheim's hypothesis elucidates that 8 
pathological process very satisfactorily, I think. Papillo- 
mata contain a matrix of embryonal cells. The keratosic 
patches undoubd^" contain, at their base, an aggregation 
of the same cells, " which amounts to a matrix.'' In 
xeroderma pigmentosum the papillomata are situated in a 
diseased area and likewise the keratosic patches. These 
if adequately supplied with vessels, as a matter of fact, 
do and are at all times liable to, give rise to carcinoma; 
more especially is this liable to occur when the tissues 
in the vicinity of the above growths are suffering from 
disease or traumatism. They are benignant by virtue 
of the physiological resistance presented by the tissues 
in their immediate vicinity though any interference with 
the normal functions of these parts may, "as was the case 
in some of those reported," cause an obliteration of the 
capillaries, and subsequent atrophy and disappearance of 
the papillomata. 
Treatment.-The method to which I would pin my faith, 
were I called upon to treat a case of this disease, is igni- 
puncture, using the galvano-cautery with medium-sized 
needle; thoroughly puncturing the spots at their periphery 
and from their centre to their periphery. Possibly in the 
case of the large verrucuous plaques the pointed cautery 
of Paquelin would be more suitable and effective. It is 
my belief if the desease be treated on its first appearance 
by this method, it will end its career there and then. 
I am not aware that it has ever been tried, though in 
the report of Dubois-Havenith's case, it is stated that he 
treated the raspberry-sized tumors by cautery and abla- 
tion; and they returned in most instances. ''However no 
mention is made of the method, or extent to which it was 
carried." 
241 DeSoto Street.