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Cullen TS. Embryology, anatomy, and diseases of the umbilicus together with diseases of the urachus. (1916) W. B. Saunders Company, Philadelphia And London.

Umbilicus (1916): 1 Umbilical Region Embryology | 2 Umbilical Region Anatomy | 3 Umbilical New-born Infections | 4 Umbilical Hemorrhage | 5 Umbilicus Granuloma | 6 Omphalomesenteric Duct Remnants | 7 Umbilicus Abnormalities | 8 Meckel's Diverticulum | 9 Intestinal Cysts | 10 Patent Omphalomesenteric Duct 1 | 11 Patent Omphalomesenteric Duct 2 | 12 Bowel Prolapsus at Patent Omphalomesenteric Duct | 13 Abdominal Wall Cysts by Omphalomesenteric Duct Remnants | 14 Omphalomesenteric Vessels Persistence | 15 Umbilical Inflammatory Changes | 16 Subumbilical Space Abscess | 17 Umbilicus Paget's Disease | 18 Umbilicus Infections | 19 Umbilicus Abnormalities 2 | 20 Umbilicus Fecal Fistula | 21 Umbilicus Round Worms | 22 Umbilicus Foreign Substance Escape | 23 Umbilical Tumors | 24 Umbilicus Adenomyoma | 25 Umbilicus Carcinoma | 26 Umbilicus Sarcoma | 27 Umbilical Hernia | 28 The Urachus | 29 Congenital Patent Urachus | 30 Urachus Remnants | 31 Urachal Remnants Producing Tumors | 32 Large Urachal Cysts | 33 Anterior Abdominal Wall Abscesses | 34 Urachal Cavities | 35 Umbilicus Acquired Urinary Fistula | 36 Urachal Concretions and Urinary Calculi | 37 Urachus Malignant Changes | 38 Urachus Bleeding into the Bladder | 39 Patent Urachus Tuberculosis | Figures
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Chapter XVII. Paget's Disease of the Umbilicus

Fox and MacLeod's case.

Milligan's case.

The results with radium in a case of Paget's disease of the umbilicus.

Eczema of the umbilicus.

The first case of this character found in the literature is that recorded by Fox and MacLeod and published in 1904. In 1911, W. A. Milligan reported a case. As the condition is very rare, these cases will be cited here somewhat in detail.

A Case of Paget's Disease of the Umbilicus.* —

The patient under consideration appeared before the Dermatological Society of London on November 13, 1901, and a microscopic section of a portion of the diseased tissue was demonstrated. At the meeting of the society on March 12, 1902, further microscopic specimens were exhibited, confirming the diagnosis of Paget's disease. The following detailed account is given by Fox and MacLeod :

" The patient, a seafaring man of sixty-five years, came under the care of Mr. W. Turner, surgeon to the Dreadnought Hospital at Greenwich, and assistant surgeon to the Westminster Hospital. The man possessed a good constitution, and there was nothing of moment to note in his personal history, and no family historj- of cancer. In the umbilical region was a rounded, eczematoid patch of about two inches diameter [Fig. 155] which had gradually been forming for about eleven years, but the applicant had not been much bothered by it, and exact details as to the history of the patch were not forthcoming. The central part of the patch was of a brilliant red color, exulcerated, and exuding serum, but silvered over in spots with epithelium. This raw center passed peripherally into a well-marked, raised, smooth, broad border, which terminated abruptly, and over which the cuticle was intact. The whole of the patch felt considerably infiltrated.

"Mr. Turner was struck by the objective features of the patch, and by its chronicity and steady eccentric progression. The man was under treatment for a considerable time, and as the patch proved quite intractable to all treatment tried short of destruction or removal, Mr. Turner very kindly brought the patient to the Skin Department of the Westminster Hospital, with the suggestion that the case was one of Paget's disease. Histologic examination after a biopsy confirmed the diagnosis, and thereupon Mr. Turner removed the diseased skin, and was kind enough to hand it to us for investigation and to allow us to record the case.

"Histologic Changes Present in the Case. — As the whole of the diseased patch was excised, an abundance of material was obtained for purposes of histologic examination. A quadrant of the excised tissue was cut out, and from this, longitudinal sections were made. As a reference to the above clinical description will show, the patch was roughly circular and had a clearly defined

  • Fox and MacLeod: Brit. Jour. Dermatol., 1904, xvi, 41.

268


paget's disease of the umbilicus. 269

raised border and an excoriated central portion. The sections of the quadrant thus included the border and the healthy tissue outside it and a portion of the central excoriated area. These sections were about an inch in length. The tissue was fixed and hardened in alcohol, embedded, and cut in paraffin, and the sections were stained with various dyes, such as borax-methylene-blue, polychrome-methyleneblue, safranin, and water-blue, to demonstrate the finer structure of the cells of the epidermis, the pseudococcidia, and the cellular and fibrous elements of the corium.

" 1. Changes in the Epidermis. — With the low power the epidermis of the outer extremity of the section showed a slight proliferation in a downward direction by a regular elongation and widening of the interpapillary processes and a rounding of their extremities. This proliferation became very much more pronounced in the middle third of the section, which corresponded to the raised edge. Here the processes had become twice the length of those in the outer third, and were far more irregular in their shape and width. Some were clubbed at the extremities, others broad and rounded, and a few were conic and tapered. Here and there, owing to the obliquity of the section of the ridge-net system, the familiar appearance of irregular islands of the corium situated in the epidermis was produced. But in spite of the irregularity in shape and size of these interpapillary processes, they all ended at about the same level in the corium, and did not spread down irregularly into it as in condyloma and epithelioma. In the outer two-thirds of the section the epidermis had an imperfect stratum corneum, which showed a tendency to desquamate and was unusually thin. Here and there it extended down in small plugs or formed concentric horny pearls where a depression existed on the surface. The basal layer was present in this situation, and although it was not perfectly regular, still it remained unbroken. The epidermis did not stain regularly, and the lower ends of the processes especially stained faintly as if they were edematous. Irregular spaces were present in the Malpighian layer, but the interepithelial lymphatics were not uniformly distended with edematous fluid as they are in psoriasis and eczema. Another peculiar feature of the epidermis noticeable with the low power was the presence in it of a number of darkly stained, more or less rounded bodies, some of which were several times larger than a prickle-cell. These were irregularly distributed in the epidermis, some being situated superficially near the horny layer, others deep down toward the basal layer, but the majority being in about the middle of the epidermis. These were arranged singly or in clusters, and occasionally they were grouped together in a concentric manner, forming variously shaped figures. They were situated among the prickle-cells, and only a few of them could be detected at the edges or lying free in the irregular spaces already referred to. These rounded structures are the "cocciclia" of Darier and Wickham.

"Toward the middle of the section the ordinary epidermis stopped abruptly, and was replaced by a single layer of columnar epithelium, which extended over the surface and dipped down at intervals to form a lining for a number of glands similar in appearance to Lieberkuhn's follicles of the small intestine. These follicles extended down into the underlying fibrous stroma, and some of them reached to a lower level than the longest interpapillary process. This showed that in this case a portion of Meckel's diverticulum had been included in the umbilicus, an occurrence which occasionally takes place. A reference to [Fig. 156] will serve to show the general appearance of the section as seen under a low power. Only a portion (about


270


THE UMBILICUS AND ITS DISEASES.


three-fifths) of the section is there depicted, the outer fifth and inner fifth bein^ left out in the drawing.



Fig. 155. — Paget's Disease of the Umbilicus. (After Fox and MacLeod.) The umbilicus as such is not recognizable, but its site presents a somewhat worm-eaten appearance. For the histologic

picture see Figs. 156 and 157.


Fig. 156. — Paget's Disease of the Umbilicus. Histologic Appearaxces ix Fig. 155. (After Fox and MacLeod.) Drawing of the central three-fifths of one of the longitudinal sections referred to in the text. It shows the raised border and the central mucous portion, a, a. Imperfect stratum corneum; b, proliferating epidermis; c, small cornified cell-nest; e, columnar epithelium lining the surface, the remains of Meckel's diverticulum; /, tubular glands lined with columnar epithelium: g, dense infiltration, consisting chiefly of plasma-cells; h, dilated blood-vessel. [This has been reduced so much in size that the finer details are lacking. — T. S. C]


" With the high power (Oc. iv, Obj. T V, Oil imm., Leitz) the explanation of the peculiar changes in the epidermal cells already referred to was apparent. Even at the outer margin of the section, but far more marked toward the center, the prickle


paget's disease of the umbilicus. 271

cells at the lower parts of the interpapillary processes were found to be swollen, their protoplasm faintly stained, and their nuclei frequently situated in spaces within the cells. The cells were evidently edematous, and though toward the surface they stained more naturally, yet the edema was still present sufficiently to interfere with the process of cornification, and there were scarcely any cells in the position of the granular layer in which even a trace of keratohyalin could be detected. The stratum lucidum was also absent, and the horny layer was unusually thin and tended to desquamate. The cornification thus took place without the formation of keratohyalin, as it does in the red portion of the lips [Fig. 157]. In spite of the edema of the cells, however, a number of nuclei in the process of




a


Fig. 157. — Paget's Disease op the Umbilicus. (After Fox and MacLeod.) Drawing of a portion of the epidermis with the raised border of the umbilical growth seen in Fig. 155. a. Pricklecells; b, edematous cell, partially cornified and globular, prickles lost, protoplasm homogeneous, granular center through degeneration of the nucleus; cell much swollen; c, cell similarly affected with edema, and showing a hardened ectoplasm with an edematous nucleus; d, multinuclear edematous cell; e, multinuclear edematous cells - one of the nuclei has become surrounded with protoplasm, forming a round cell.

karyokinesis were observed, and the cells of the basal layer and those immediately above it showed numerous mitotic figures.

" The inter epithelial edema was not pronounced in the middle and upper portion of the epidermis, though here and there it was sufficient in degree to allow of leukocytes making their way between the cells toward the basal layer. Wide, irregular spaces were present, in which were deformed prickle-cells, leukocytes, and debris. A number of prickle-cells were found to have lost their fibrillary skeleton, the spongioplasm and its continuations into interepithelial fibrils had disappeared, and the protoplasm had become homogeneous. In this way the cells had assumed a globular appearance. Many of these cells lying immediately beneath the stratum corneum had become surrounded by a hardened, probably keratinized, covering.

"Several types of these degenerated cells were formed in this way, and these were variously grouped, e. g.:

" (a) Round, swollen cells with a finely granular, almost homogeneous proto


272 THE UMBILICUS AND ITS DISEASES.

plasm, and a darkly stained nucleus lying in a space or surrounded by a halo of fluid protoplasm, which stained faintly.

  • ' These nuclei had chromatin bodies and a good intranuclear network.

" (b) Round or oval cells with a faintly stained nucleus, but a more defined and darkly colored ectoplasm, which stained similarly to that of the cells of the stratum corneum. These cells had a slight resemblance to coccidia.

" (c) Cells in which, in spite of the edema, an active nuclear division had taken place, but in which the division of the protoplasm of the cell had not kept pace with that of the nuclei, and so multinucleated cells containing several oval, faintly stained nuclei had been produced.

" (d) Groups of cells in which the nuclei had become flattened and crescentic in form, and a great variety of shapes had resulted. It is unnecessary to describe in detail these different groups and figures. Occasionally a leukocyte had become impacted in such a group and further complicated it.

"The single cells, or 'pseudococcidia, ' could be demonstrated by any of the ordinary stains, such as methylene-blue, hematoxylin, and picric acid (Banti), but the most satisfactory specimens of them were obtained by staining the protoplasm of the cell with water-blue and the nuclei with safranin.

"The columnar epithelial cells lining the surface of the central portion and the follicles which dipped down from it were seen under the high power to be very regular in shape, and to have oval nuclei situated near the base of the cell. These cells appeared to be perfectly healthy, and showed no evidence of edema or other degenerative process.

"2. Changes in the Corium. — The most noticeable feature in the corium when examined under the low power was a dense sheet of cellular infiltration, which occupied the papillary and subpapillary layers and the upper portion of the reticular layer. This infiltration was densest in the middle third of the section, especially where the raised border existed, and in this situation it was peculiarly diffuse and ended abruptly below in an almost straight line. It was not quite so dense in the papillae, and about the blood-capillaries the cells tended to be collected in foci. At the outer end of the section it was less diffuse, and was arranged in foci around the papillary and subpapillary blood-vessels, while in the center, beneath the columnar epithelium, it was also less dense and more irregular, and spread farther down into the underlying stroma.

" With the high power the infiltration was found to consist largely of plasma-cells, with a few leukocytes and connective-tissue nuclei. These plasma-cells were perfect in shape and showed no tendency to special grouping or to form giant-cells. This cellular infiltration was thus more than a simple inflammatory infiltrate, such as is met with in eczema, psoriasis, or any acute inflammatory condition of the skin. It was more closely allied to that which occurs in certain of the 'infective granulomata, ' such as syphilis and yaws, and suggested a chronic inflammatory process. Unna described it as a singularly pure 'plasmoma, ' and Karg has likened it to a bulwark against the cancerous invasion.

" The papillae were edematous and swollen, especially in the middle of the section. The fibrous elements of the corium were affected only in the area of infiltration. There the collagen stained faintly, especially in the edematous papillae, but showed no basophilic degeneration. The elastin was also affected in that it stained badly, was swollen, and formed an imperfect supporting skeleton.


paget's disease of the umbilicus. 273

The blood-vessels of the papillary and subpapillary layers were much dilated, and there were a few dilated capillaries in the corium beneath the infiltration."

(A brief resume of the literature of Paget's disease follows.)

Remarks on the Histology of our Case and Conclusions. — There are several points of interest in connection with the microscopic changes present in our case which, although they can hardly be said to settle this controversy, still are worthy of consideration:

"1. Although the affected epidermis was that of the umbilicus and not the areola of the nipple, still, the changes present in it, the peculiar degenerated pricklecells, the occurrence of the dense sheet of plasma-cells infiltrating the underlying papillary layer of the corium, in short, the whole histologic architecture, was similar in every detail to that which has been repeatedly described in the typical cases of the disease. These initial peculiar cellular changes in the epidermis, allied somewhat to those which occur in Psorospermosis follicularis vegetans (Darier's disease), could no longer be mistaken for those of chronic eczema or psoriasis, and it is unnecessary to repeat any labored details with regard to the histologic diagnosis from these affections. It would seem that the histologic changes in the epidermis in Paget's disease are characteristic and pathognomonic, whether the affection occurs in the nipple, the umbilicus, or the genitalia.

"2. In this case there was no evidence of definite malignant change in the epidermis. The degree of proliferation was limited, and the basal layer was intact. It has been asserted that the peculiar change of the epidermis is malignant from the first. This does not seem to us to be so any more than that ordinary warts, the warty growth in xeroderma pigmentosum, or pigmented nevi (moles), are malignant from the outset. They may all be described as precancerous lesions of the skin which have a potentiality more or less certain of becoming malignant.

"3. The inclusion of a portion of Meckel's diverticulum in the center of the umbilicus, in this the only case of Paget's disease which has been recorded in that situation, may be a coincidence, but it is a suggestive one. Cases have been recorded in which the cancer grew from the epithelial cells of mucous glands, and, had malignancy supervened, it is possible that it might have taken its origin in the cells lining the follicles in the cut-off portion of gut in the umbilicus. Still, in the sections the columnar epithelium on the surface and lining these follicles seemed perfectly healthy, although the neighboring epidermis was markedly affected."

[The causative factor in Fox and MacLeod's case is clearly evident. From Fig. 156 it will be seen that some of the tubular glands which were similar to those of the small intestine opened directly on the surface, and naturally produced some secretion which would keep the parts moist and tend to irritate them. The nature of the man's occupation favored lack of systematic bathing. During early and middle life nature was able to resist any active cell changes, but when he reached the period at which atypical cell changes are prone to occur, the first symptoms manifested themselves. From the history it is seen that he was fifty-four when this process was first noted, and that it had gradually increased until he came under observation eleven years later.

In the case reported by Milligan, and later by Pinch, the patent urachus was evidently the exciting factor. It is particularly interesting that in both of the recorded cases the cause was a congenital umbilical defect. — T. S. C] 19


274 THE UMBILICUS AND ITS DISEASES.

Pa get's Disease of the Umbilicus Cured by the Application of Radium.* — " Mrs. W., aged thirty-one, came complaining of a smelly discharge from the navel, accompanied by an eruption around the navel. The trouble had begun four years previously, with a smarting pain around the waist and a redness toward the right side of the umbilicus.

"Ordinary remedies were tried, but with no success, the condition steadily getting worse. The patient was then subjected to x-ray treatment — four applications of ten minutes each. This apparently cured it, but very shortly afterward it broke out again. For twelve months or so ordinary remedies were resorted to, but with no result. Again .r-ray treatment was tried, — six applications, — but this time it got worse instead of better.



Fig. 158. — Paget's Disease op the Umbilicus. (After Milligan.) The small opening in the umbilicus is clearly seen. Surrounding this is a granular, sharply circumscribed, raised area, involving the abdominal wall on all sides. The appearance of the umbilicus after the use of radium is seen in Fig. 159.

"Sir Malcolm Morris saw the patient in consultation about the middle of June last, and he advised either total excision or radium treatment. Accordingly, small doses of radium were applied around the edge of the eruption, which now had a radius of about two inches from the umbilicus. The radium was applied in successive places around the edge, and each place had an exposure of four hours. This certainly had a good effect, although it did not cure it. Finally, on August 21, 1911, at the Radium Institute, the patient had a treatment of 70 mg. of pure radium for one and one-half hours direct on the skin, there being no intervening screen. For ten days nothing was felt by the patient, and then she had a burning sensation around the waist, and the discharge got worse. This lasted for two weeks, and then the skin healed, leaving only a small sore spot on the right side.

"The condition prior to the last application of radium is well shown in the

  • Milligan, W. A.: Proc. Roy. Soc. Med. (Dermat. Section), November, 1911, v, No. 2, 30.


PAGET S DISEASE OF THE UMBILICUS.


275


photograph [Fig. 158], and consisted of a raised, indurated edge all around, with a raw weeping surface extending into the umbilicus.

"The condition is now apparently cured [Fig. 159], although there is still some discharge, and the question arises as to whether there may or may not be a patent urachus. This has not been conclusively proved, although at times the discharge has an ammoniacal smell. It is interesting to note the large close of radium used by Mr. Pinch at the Radium Institute, a dose corresponding to 2,000,000 activities."

Mr. A. E. Ffayward Pinch, when referring to the same case, said that a slight



Fig. 159. — The Appearance in a Case of Paget's Disease of the Umbilicus after Treatment with Radium.

(After Milligan.) The umbilicus is relatively smooth, but somewhat paler than the surrounding tissue. The line of demarcation of the tumor is still clearly evident. The skin around the umbilicus looks normal, but to the (patient's) left there apparently is still a little thickening. For the appearance of the umbilicus before treatment see Fig. 15S.


recurrence took place early in September, 1912. The same treatment was adopted, with an equally good result, and the patient since then had remained perfectly well.

Sir Malcolm Morris, chairman of the meeting, said that a case of Paget's disease of the umbilicus was shown years ago before the old society by Mr. Marmaduke Sheild.

In 1912 I wrote Dr. Milligan asking if it would be possible for him to send me photographs of his case, as the reproductions in the Proceedings of the Royal Society were not very satisfactory. Dr. Milligan complied with my request and sent me the photographs here reproduced.


276 THE UMBILICUS AND ITS DISEASES.

ECZEMA OF THE UMBILICUS.

This condition is by no means rare, although the literature on the subject is very meager. In the new-born, during the process of cicatrization of the cord, there may be slight irritation of the umbilicus without any evidence of infection. In an adult with a very delicate skin there may be a slight irritation of the umbilicus and some cracking of the skin, notwithstanding the utmost cleanliness and care. This is prone to occur in stout individuals when the weather is excessively warm and the patient perspires a great deal. The most common cause of an eczematous condition around the umbilicus is the existence of an umbilical concretion, which, on account of the contracted condition of the umbilical opening, is frequently overlooked. Cantrell.* in 1897, and Morris, f in 1895, briefly discussed eczema of the umbilicus. Umbilical concretions are discussed in detail on p. 247.

Recently I saw a mild case of eczema of the umbilicus in consultation with Dr. Frank Sladen in the Johns Hopkins Hospital. The patient was eighteen years old. From time to time there had been an irritating discharge from the umbilicus. On examination I found an eczematous condition in this situation. There was a little depression at the side of the umbilical depression. There was no evidence of a concretion.

  • Cantrell, J. A.: Eczema Umbilici and its Treatment. Therap. Gaz., 1897, xxi, 82.

" Morris. R. : Lectures on Appendicitis and Notes on other Subjects, 1895, 93.



Umbilicus (1916): 1 Umbilical Region Embryology | 2 Umbilical Region Anatomy | 3 Umbilical New-born Infections | 4 Umbilical Hemorrhage | 5 Umbilicus Granuloma | 6 Omphalomesenteric Duct Remnants | 7 Umbilicus Abnormalities | 8 Meckel's Diverticulum | 9 Intestinal Cysts | 10 Patent Omphalomesenteric Duct 1 | 11 Patent Omphalomesenteric Duct 2 | 12 Bowel Prolapsus at Patent Omphalomesenteric Duct | 13 Abdominal Wall Cysts by Omphalomesenteric Duct Remnants | 14 Omphalomesenteric Vessels Persistence | 15 Umbilical Inflammatory Changes | 16 Subumbilical Space Abscess | 17 Umbilicus Paget's Disease | 18 Umbilicus Infections | 19 Umbilicus Abnormalities 2 | 20 Umbilicus Fecal Fistula | 21 Umbilicus Round Worms | 22 Umbilicus Foreign Substance Escape | 23 Umbilical Tumors | 24 Umbilicus Adenomyoma | 25 Umbilicus Carcinoma | 26 Umbilicus Sarcoma | 27 Umbilical Hernia | 28 The Urachus | 29 Congenital Patent Urachus | 30 Urachus Remnants | 31 Urachal Remnants Producing Tumors | 32 Large Urachal Cysts | 33 Anterior Abdominal Wall Abscesses | 34 Urachal Cavities | 35 Umbilicus Acquired Urinary Fistula | 36 Urachal Concretions and Urinary Calculi | 37 Urachus Malignant Changes | 38 Urachus Bleeding into the Bladder | 39 Patent Urachus Tuberculosis | Figures

Reference

Cullen TS. Embryology, anatomy, and diseases of the umbilicus together with diseases of the urachus. (1916) W. B. Saunders Company, Philadelphia And London.

Historic Disclaimer - information about historic embryology pages 
Mark Hill.jpg
Pages where the terms "Historic Textbook" and "Historic Embryology" appear on this site, and sections within pages where this disclaimer appears, indicate that the content and scientific understanding are specific to the time of publication. This means that while some scientific descriptions are still accurate, the terminology and interpretation of the developmental mechanisms reflect the understanding at the time of original publication and those of the preceding periods, these terms and interpretations may not reflect our current scientific understanding.     (More? Embryology History | Historic Embryology Papers)

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