Part 2

CASE 8.—Charles C——, aged 32 years, tailor, came to University College Hospital on October 4th, 1904. He stated that the skin lesions had commenced four years previously, appearing first on the thighs, then on the legs, and a little later on the forearms. He did not remember when the body was first affected. Fresh patches have appeared from time to time, but none have gone away. The lesions consisted of irregular patches from half to several inches in diameter symmetrically distributed over the trunk and limbs. On the back, they were in elongated or oval patches, symmetrically arranged in oblique lines corresponding to the direction of the ribs. They are well defined from the healthy skin, not raised above the normal skin, and the border was not raised above the central portion either to sight or touch. In front, there were large areas due to coalescence of several patches, but the smaller patches were elongated and horizontally placed. On the thighs near the groin the patches ran obliquely round the limb, but lower down were vertical. The general colour was yellowish pink, but in some the yellow, in others the pink predominated. When pinched up, many of the patches were distinctly thickened, but in the more recent this could not be felt, and some appeared as mere stains. The surface was smooth on the trunk, but on the lower limbs there was some roughness of the surface, more marked below the knee, but not actually scaly. The patches on the trunk were larger than on the limbs, and healthy areas of skin were sometimes completely enclosed by the diseased patches. The upper part of the chest, neck, and face were free, except a small patch on the lower lip. There were also large spaces of unaffected skin on the thighs, but only small ones on the upper limbs. On the back of the hands near the wrists, there were some small patches. The mucous membrane of the mouth was normal. There was no itching nor other subjective sensation. There was a history of phthisis in his mother and brother, but the patient was well nourished and healthy, except that he was liable to bronchitis, and had some enlarged post sterno-mastoid glands on the left side. He was put upon salicin gr. xv _ter die_, and vasogen iodine rubbed on the forearms, but so far no decisive result has been obtained. Mr. Pernet has obtained a piece of skin from the right forearm, where the lesion was apparently superficial. His report is appended.

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CASE 9.—A gentleman, aged 29 years, in whom the disease has been present for four and a half years, was seen with Mr. George Pernet, who showed him at the Dermatological Society of London in November, 1904, and the notes of his case were published in the December number of the _British Journal of Dermatology_, vol. xvi, p. 457.

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There remains only the case of the lady, which requires separate consideration.

* * * * *

CASE 10.—Mrs. H—— was sent to me by Dr. Vassie on January 18th, 1902. She had suffered from the disease for nine or ten years, had seen other dermatologists, and visited sulphur and other spas. The disease had begun with a single patch on the arm, and after remaining single for a few months, had spread and extended almost all over the body and limbs.

When I saw her, the lesions were practically all over the trunk and limbs with ill-defined pale red patches with powdery roughness and decided thickening of the whole cutis of the patches, most marked in a 3-inch patch, situated high up on the right loin. The thigh patches were also thickened, but on the arms and forearms the thickening was less marked. On the front of the legs, the patches were irregular and more decidedly scaly, but not like either eczema or psoriasis. The eruption, as a whole, was like a seborrhoic eczema but less defined, and in parts the lesions were very pale and shaded off into the healthy skin. The neck, face, and hands were free. It smarted in cold weather, but did not itch.

The catamenia were regular, and her general health when seen was excellent, but a year previously she had had a fibroid removed after it had begun to be troublesome for a month from hæmorrhage. Before that the catamenia were twice a month. There was slight seborrhœa capitis. Salicin, 15 grains three times a day, was given. On July 17th she was seen again, and the eruption on the legs was somewhat paler, and that on the arms was much paler, but over the scapula the lesions were thickened but paler; but she attributed the improvement to the warmer weather during the last month, as there was generally some improvement in the summer, while in the winter it fell back, cracked, and smarted. She was not seen again until May 12th, 1904, having meanwhile had other advice, but without any improvement, and none of the patches had gone away. At Wiesbaden she had subcutaneous injections of cacodylate of soda in the month while she was there, and once a fortnight for twelve weeks after, but without benefit. It was noted that the leg’s were distinctly red and scaly. On the rest of the limbs and body the patches were red and scaly but without thickening, except on the forearms, the patches on the left being distinctly thickened. The patches were large, many being palm-sized and in the aggregate covered three fourths of the body, but the face and hands were quite free, and the neck nearly so. There was no itching. As every previous treatment had failed, the possibility of the disease developing into Mycosis fungoides being entertained, although itching was absent, it was resolved to try the effect of the Röntgen rays on a portion of one leg. Nine exposures of ten minutes each at a distance of eight inches were given, and a month after there was slight improvement where the rays had been used, but it was not very decided; but on the left arm where vasogen iodine had been rubbed in there was decided improvement, the lesions being pale, less scaly, and there was some clearance as compared with the right side, which had not been treated. I did not see her again until February 7th, 1905, and she then told me that the improvement where the X-rays had been applied had continued, and in a few weeks after I last saw her, in August, 1904, the disease had entirely disappeared from the outer side of the leg below the knee, and the skin when I examined it was quite white and smooth on the part exposed and had been so for four months, while the inner side of the leg where the rays had not reached remained unaltered. She had not continued the vasogen iodine to the arm, and it had become scaly again.

General Remarks on the Disease.

From the above description and the cases related in support of it, what inference may be drawn as to the nature of the disease and its relation to other skin affections? Frankly, at present I am quite unable to even conjecture its pathology, or to suggest, with one exception, any relation to other dermatoses. In my first cases I supposed that it was an early stage of Mycosis fungoides, though the absence or trifling character of the subjective symptoms did not lend this any support. I clung to this theory, however, _faute de mieux_, for a long time, but it received its _coup de grace_ when I met with a case of over thirty years’ duration without any such malign development.

At the Dermatological Society of London, where Mr. George Pernet and myself have shown three cases, among other suggestions, that of a possible seborrhoïde or Urticaria pigmentosa have been made. Against the former, the absence of scaliness in a large proportion of the patches, that the patches are in the skin, not raised above, and that itching is an exceptional feature, together with the unchanging character of the lesions, effectually bar the diagnosis of a seborrhoïde. Neither can I find anything beyond the yellowish tint frequently, but not always, present to support the idea of Urticaria pigmentosa. The absence of itching in nearly all the cases of Urticaria factitia, while the patches are level with the normal skin, and their unchanging character, are all strongly against such a diagnosis, to say nothing of the extreme rarity of adult Urticaria pigmentosa and the improbability of one man meeting with nine cases of it. The histology also negatives this.

I can therefore, at present, only regard it as a dermatosis _sui-generis_, of which the pathology must be left an open question, as Mr. George Pernet’s report on the histology of the portion of skin removed from Case 8 does not throw much light upon the pathology. There remains only the affection described by Brocq, to which, certainly, there are closer resemblances than to any other affection.

Brocq[1] divides the group which he calls parapsoriasis into three groups:

First variety (very closely related to psoriasis), Parapsoriasis guttata. Jadassohn’s case is probably to be referred to it.

Second variety (intermediate between Lichen and psoriasis), Parapsoriasis lichenoides, including Parakeratosis variegata of Unna, and Lichen variegatus (Crocker).

Third variety (closely allied to Seborrhœa psoriasiformis), Parapsoriasis in patches, corresponding to Erythrodermie pityriasique en plaques disseminées of Brocq, and of which cases have also been reported by J. C. White and C. J. White.

It is only with the third variety that comparison need be made, the deep colour and very small pattern, like a mosaic of the first two, sharply contrasting with the broad effects as of colour dashed on, in Xantho-erythrodermia perstans.

For the whole group Brocq gives the following characteristics:

(1) An almost complete absence of pruritus.

(2) A very slow evolution.

(3) A distribution in circumscribed, sharply defined patches, whose dimensions are from 2 cm. to 6 cm. in diameter, and which are scattered here and there over the integument.

(4) An almost complete absence of infiltration of the derma.

(5) A pale redness (pinkish coloured).

(6) A fine pityriasic desquamation.

(7) An extraordinary resistance to the local applications usually employed in the treatment of psoriasiform or pityriasic seborrhœa, in fact, only yielding slowly and imperfectly to the most energetic application of pyrogallic acid.

The special features of the third variety he describes as:

(1) Being in patches, circumscribed, sharply defined from 2 cm. to 6 cm. in diameter.

(2) They are scattered irregularly over the skin without any apparent system.

(3) The colour varies from a pale red to a brownish or livid red, according to the part affected.

(4) There is always present a fine pityriasic desquamation more or less marked in different cases.

(5) There are at times in some of these cases, aggregations of small flattened papules which may be considered as links connecting it with the second variety.

(6) There is no infiltration of the integument appreciable to the eye or touch.

(7) The face is rarely affected, and there is the same extremely slow evolution, great resistance to local treatment, and few or no subjective symptoms.

Referring to the whole group, he says: “We know nothing very definite about the etiology or pathology of these affections. They may appear at any age, but seem more frequent in youths or adults. He has observed cases in men and women and in all classes of society. They seem to be slightly more frequent in women than in men.”

It must be confessed that there are many points of resemblance of this third variety to Xantho-erythrodermia perstans, and, as regards the lady, Case 10, it is probably what Brocq has described as “Erythrodermie pityriasique en plaques disseminées,” although I should say there was distinct infiltration in a large proportion of the lesions, and many of the patches far exceeded the limits in size that Brocq lays down.

With regard to the other nine cases, the differences are somewhat more marked, and they should, at least for the present, be either kept apart or treated as a distinct variety.

The differences are:

(1) The patches are frequently much larger, 3 and 4 inches or more in their long diameter, and the margin is not very sharply defined.

(2) A distinct arrangement in lines in direction varying with the topography is observable in most cases.

(3) The colour is either pale red or distinctly yellowish.

(4) Instead of a fine pityriasic desquamation being always present, this is only distinct on the legs, sometimes just recognisable on the thighs and arms, while on the trunk it is absent, the surface being usually quite smooth.

(5) The presence of papules I have not observed, except at the commencement of Case 4.

(6) There is distinct infiltration in a large proportion of the patches, perceptible to the touch, though not to the eye.

The resemblances are: the absence of conspicuous pruritus, and there is often none; the occurrence in patches; the slow evolution; a pale redness; in many cases an extraordinary resistance to treatment. These are not enough to establish identity, but I confess that in my opinion they show greater resemblance to the affection I am describing than they do to psoriasis, and certainly my nine male cases would never suggest to any one a resemblance to psoriasis, and I should strongly demur to class them under parapsoriasis as a covering term.

Histological Note by George Pernet.

A piece of skin was removed from the extensor surface of the right forearm of the male case, aged 32 years (a Jewish tailor), where the more recent patches had appeared. Clinically there was practically no infiltration to be felt in that situation. The specimen was hardened in alcohol, cut in celloidin, and stained in various ways. The microscopical appearances were as follows:

_Epidermis._—The stratum granulosum was either atrophied or absent. The stratum lucidum was absent except for traces here and there. The epidermis generally showed a slight amount of œdema.

_Corium._—The vessels were dilated, with some cellular infiltration about them. The collagen appeared to be normal, with the exception of slight œdema, but the elastin was apparently reduced in quantity, especially in the papillary layer, and it was to some extent fragmented in places. It should be mentioned that the specimen was stretched on a small piece of cork in the process of hardening. The elastin stained, however, much as in the normal condition.

Altogether there was little to be gathered from the histology _quâ_ cause, except that the appearances of the blood-vessels perhaps pointed to a general blood condition.

Dr. Thiele, Pathologist to University College Hospital, kindly examined the blood and reported: Total red corpuscles per c.mm., 6,560,000; total whites per c.mm., 21,878. Hæmoglobin, 84%. C.T., 65. Differential count of whites: Small lymphocytes, 18·2%; large lymphocytes, 9·4%; neutrophiles, 60·7%; oxyphiles, 1·6%: hyaline cells, 2·1%.

Footnote 1:

_Amer. Journ. Cut. Dis._, vol. xxi, 1903, p. 315.

IDIOPATHIC MULTIPLE PIGMENT SARCOMA (IDIOPATHIC MULTIPLE HÆMORRHAGIC SARCOMA) OF KAPOSI. By F. PARKES WEBER, M.D., F.R.C.P., AND PAUL DASER, M.D.

The patient, Jakob Z——, is a Polish Jew, aged 46 years, from Galicia, who has lived nine years in England, and says he has enjoyed good health. There is no evidence of previous alcoholism or syphilis. The patient was brought by Dr. Weber before the Dermatological Society of London on February 8th, 1905.

The present illness commenced about three years before, when he chanced to wound the sole of his right foot with a nail. A pedunculated growth, about the size of a cherry, arose from the wound. This description reminds one of the strawberry-like granulation tumours sometimes growing from small wounds or ulcers on the fingers, which have been termed “botryomycosis,” or rather “botryomycomata,” because at one time they were supposed to be caused by the “botryomyces.”[2] The growth was removed by a doctor. Afterwards small bluish nodules developed from time to time on the feet and legs. Some of these nodules apparently undergo the following series of changes: They slowly increase in size, and after about three months constitute little pendulous tumours, which ultimately fall off spontaneously or else are knocked off or pulled off by chance; in this way local healing occurs, but fresh nodules form elsewhere. Though, as stated, some of the nodules become pedunculated growths, others appear to atrophy and merely leave brownish pigment in the skin, whilst others seem to undergo very little change, and, at all events, persist for a long time in their primitive form, namely, as hard bluish lumps under the epidermis. Treatment has been as yet without result.

_Present condition_ (January, 1905).—On _the hands_ about the knuckles and backs of the fingers are a few minute slightly elevated bluish nodules. On the sole of the _right foot_, especially on the inner part, there are nodules in various stages of development. The minute ones, those in an early stage of development, are slightly elevated and of a bluish colour, similar to those on the hands. Others, further advanced in development, are small, sessile, or pedunculated hard outgrowths, without bluish colour and sometimes covered by thickened epidermis. There is a large patch of pigmented (brown) skin on the dorsal surface of the right foot, and in the neighbourhood of this pigmented area are some minute nodules of similar character to those already described. It is possible that the pigmented skin has been the site of minute growths which have undergone spontaneous involution, and have, as frequently happens in Lichen planus, left pigmentation of the skin behind them. The right thigh and leg are not affected.

The _left lower extremity_ is the part most affected by the disease, and there is chronic, rather firm, œdema of the left foot and ankle. The nodules on the left foot are similar to those on the right, but are more widely distributed, and the left foot differs from the right in the absence of the pigmented area and in the presence of the œdema to which we have just alluded. Of the little sessile or pedunculated tumours on the back of the left foot and in the neighbourhood of the ankle-joint (see Fig. 1) the biggest are of the size of a large pea. There are likewise little tumours of various sizes—that is, in various stages of development—on the inner aspect of the left popliteal region (see Fig. 2) and in the neighbourhood of the patella, the smaller ones being bluish and slightly elevated, the larger ones being reddish, lentil-sized, sessile outgrowths.

There are no nodules or growths on other parts of the limbs, trunk, or head, with the exception of four or five minute purplish or bluish papules on the _glans penis_ and neighbouring skin.

There is no evidence of any disease of the thoracic or abdominal viscera. The patient is not strongly built, but looks well nourished. He complains of various pains, but whether these are related to the disease or not is doubtful.

_Microscopic examination._—One of the growths, of the size of a large pea, was removed from the left ankle, and a smaller one (lentil-sized) from the left knee. Sections of both were stained with hæmatoxylin and eosin-hæmatoxylin. The growths are seen to be situated in the corium, and are circumscribed. The epidermis over one of them is thickened. They consist of oval and spindle cells, which, in some parts, are more or less grouped into strands or bundles, and are cut in various directions. In some parts of the sections there are empty spaces (? lymph spaces) and in some parts there is homogeneous substance between the cells; in other parts there is extravasated blood. The cells in the larger and older of the two nodules are decidedly more elongated and fibrous-looking than in the smaller and more recently developed one. Dr. J. M. H. MacLeod is kindly going to make a further report of the microscopical features.

_Remarks._—It seems to us clear that the case is a typical but relatively early one of the so-called idiopathic multiple pigment sarcoma (idiopathic multiple hæmorrhagic sarcoma) of Kaposi and later writers. For the literature on the subject we refer to the English summaries of Dr. J. H. Sequeira[3] and Dr. Radcliffe-Crocker.[4] The patient in our case is a male, as in nearly all other recorded cases of this disease; he is of the favourite age (between 40 and 50), and it may likewise be noted that he is a Galician Jew, as the patient of Sir Stephen Mackenzie[5] and Dr. J. J. Pringle was. The minute bluish nodules in the corium may be regarded as the primary feature of the disease. They either remain for a long time without undergoing much obvious change, or they undergo spontaneous involution and completely atrophy, leaving behind them only a little brownish cutaneous pigmentation; or else (thirdly) they may increase in size, become much elevated, and form definite sessile tumours, which, later on, become pedunculated, and finally drop off. This is the cycle in regard to the development of the little pendulous tumours which constitutes one of the most characteristic features of the disease. Another characteristic feature of the disease, probably sooner or later present in every case, is the œdema which occurs in the parts most affected, especially in the lower extremities. It is persistent œdema, and results in an elephantiasis-like condition. It is well marked in the left foot and ankle of our case. The striking blue or purple colour of the small nodules (which is absent in the pendulous tumours) seems, as in other cases, to be due to the venous blood contained in the blood-vessels or extravasated. What pigment there is in the growths is probably derived from blood. In our patient there is no evidence of gout as in certain cases described by Mr. Hutchinson.[6]

The prognosis in the present case is not altogether unfavourable, considering that the patient’s general health is good, that the disease is probably seldom of itself fatal, and that in some cases improvement or spontaneous care has, apparently, occurred after the disease has lasted twenty years or more.

Footnote 2:

_Vide_ X. Delore, _Lyon Médicale_, July 16th, 1899, p. 376; J. Sabrazès and A. Laubée, _Arch. Gén. de Méd._, Paris, November, 1899, p. 515; R. von Baracz, _Wien. klin. Wochenschr._, 1901, No. 14; G. Carrière and G. Potel, _Presse Médicale_, Paris, May 17th, 1902, p. 471; and L. Legroux, “La Botryomycose,” _Thèse de Paris_, 1904. The botryomyces appears to have owed its supposed existence to a mistaken interpretation of microscopic appearances. H. Bichat (_Arch. Gén. de Méd._, February 2nd, 1904, p. 281) thinks there is nothing specific in the growths, but V. Ball (_Arch. Gén. de Méd._, August 2nd, 1904, p. 1921) concludes that that botryomycosis, though it owes its name to an error, is nevertheless a pathological entity and is a special staphylococcal affection. Dr. Weber possesses a microscopic section of one of these little growths, which was removed from the finger (close to the nail) of a woman in 1890, when he was a house-surgeon at St. Bartholomew’s Hospital for Sir William Savory. It was a typical strawberry-like “botryomycoma” with quite a narrow pedicle. Dr. J. M. H. MacLeod, who has kindly examined the section in question, tells us he regards such growths as “septic granulomata,” septic organisms producing very various effects according to their degree of virulence and naturally according to the nature of the living soil on which they grow.

Footnote 3:

_British Journal of Dermatology_, June, 1901, p. 201.

Footnote 4:

_Diseases of the Skin_, third edition, 1903, p. 963.

Footnote 5:

_British Medical Journal_, June 4th, 1890. Amongst foreign accounts _vide_ Radaeli’s report of five cases in _Lo Sperimentale_, December, 1904, p. 1023.

Footnote 6: