The above provisional clinical title was suggested to me by my coadjutor at University College Hospital, Mr. George Pernet, for a well-defined affection of the skin, of which I have met with ten instances during the last three years, all but one of them in private practice. I am not aware that the disease in question has been described before, unless it can be brought under Brocq’s “erythrodermies pityriasiques en plaques disseminÉes,” with which it will be closely compared when the cases themselves have been considered. A case which I showed at the Dermatological Society of London in October, 1904, when Drs. Hallopeau, Gastou, Jacquet and Pautrier were present, was not regarded by them as a case of Brocq’s disease, with which they were presumably familiar, but as an entirely new affection in their experience. The following description is drawn up from nine of the cases, all males, which, in the main features, closely resemble each other. The remaining case, a lady, had some important differences which will be discussed later. So far, all the cases have been adults, though some of them were young. The lesions are evolved in patches of a pale pink or yellowish hue on the limbs and trunk, the uncovered parts, such as the face and hands, being free or very slightly affected. Generally, the patches come out very gradually and in small numbers and, in the main, symmetrically, but as the older patches never go away spontaneously, while fresh ones are continually evoluting at short or long With regard to individual patches, they are usually of oval or elongated form, arranged symmetrically in oblique lines on the back in the direction of the ribs, probably in the lines of fission, more or less horizontal in direction in front, and often, but not always, in vertical lines on the limbs. On the latter, especially the thighs, they not infrequently present the appearance of streaks formed by the finger, the upper part of the stroke being abrupt, and the lower shading off. This may sometimes also be seen on the trunk. The majority of the single patches range from one to three inches in their longest diameter; the borders are not very well-defined nor raised above the rest, but there is no difficulty in discerning the morbid from the healthy skin. They are not raised above the surface, but may be rather deep in the cutis. Infiltration can often be distinctly felt when the patch is pinched up in comparison with the adjoining healthy tissues, but in the more recent and smaller patches it is imperceptible, and occasionally they look like mere stains. Their colour is either pale pink or yellowish; in some cases the yellowish hue is pronounced, in others absent or nearly so; on the lower limbs the pink hue predominates. The surface is smooth on the trunk, but is often slightly rough on the arms and thighs, and below the knees maybe distinctly rough or even in branny scales. The patches are never so marked on the upper as on the lower limbs, the palms are always free, and the backs of the hands are generally unaffected, but sometimes there are a few small patches below the wrist. The face is nearly always free, though I have seen faint patches in one case. There is very little to suggest that the disease is inflammatory, and itching is quite absent in most of the cases; a few patients said they had some itching when hot, but only in one case was it really complained of, and that only in the early evoluting stage of the patches. The initial site for the lesions varies; the thighs are the most frequently first affected, the legs next in frequency, and The duration of the disease may be very long. My first case had been developing for over ten years, others had been only for a few months; but in the case of a medical man, over 50 when I saw him, he said that patches first appeared on his legs when he was a house-surgeon, and had been slowly evolving ever since, so that after thirty years he was pretty thickly covered, as none as far as he knew had gone entirely away, though they had temporarily disappeared when he had rubbed in chrysarobin ointment, but had gradually returned to their old site. The disease is compatible with perfect health; and even when there was any departure from the normal there was no reason to suppose that the abnormality was in any way connected with the skin lesions, while the majority of the patients had above the average health for their age. While there appears to be no tendency in the disease to spontaneous involution, they are not, as the case narratives show, altogether rebellious to treatment, and in at least two cases a cure appears to have been effected and in others some improvement, while in a residue no improvement could be noted. The agents which appeared to have a good effect are salicin in 15-grain doses at least three times a day, which by itself entirely cured a recent case (Case 5) of only two months’ duration and of rather acute development, and vasogen iodine 10 per cent. rubbed in is a useful supplement and materially aided in the cure of Case 4. In some cases, salicin has failed to make any marked impression on the lesions, while in others the patient has not gone on with it sufficiently long to test its merits. As might be expected, it has been most successful when the disease has been present for a short time. The only female case, a lady aged 47 years, resembled the other cases in its gradual evolution, long duration, absence of itching, in the persistence of the old patches with continual evolution of new ones, in its limitation to the covered parts, and in the general good health of the patient. The differences were in the patches being distinctly scaly all over the body; though the scales were small and even powdery in most parts of the body, they were, as usual, rather larger and more abundant on the legs. The patches were also more decidedly red than As regards to etiology, it is chiefly negative. There is a large preponderance of males, and all the cases have been over 20 years old, while 56 years is the oldest I have met with. In no case could an exciting cause be made out; two of the patients had had syphilis, but it did not appear to have any etiological importance, and in one of them antisyphilitic treatment was tried vigorously for twelve months without effect. Case 1.—Mr. O——, draper, aged 30 years, was first seen by me on March 5th, 1902. The disease had been present ten years. From the commencement none of the patches had gone away. They appeared simultaneously inside the arms and thighs. They increased in numbers very slowly for a long time, and were confined to the limbs until three years ago, when they attacked the trunk, and during the last year have greatly increased in number; in fact, most of them have appeared in the last twelve months. His father was drowned and his mother died of fatty heart at the age of 51 years. When first seen by me the disease was in yellowish patches which commenced four inches above the nipples, but were not abundant till the line of the nipples, and they were less numerous below the umbilicus than above it. They were rather thickly arranged in horizontal elongated patches from 1 to 3 inches long, and 1/2 inch wide, as if streaked by the finger, pale, pink, or yellowish in tint, rather well-defined, but the edges were not sharp, and when the patch was pinched up a slight infiltration or thickening could be felt in the skin. The longer patches were formed by coalescence of some of the smaller ones. The surface was quite smooth. On the sides, the patches inclined slightly downwards and forwards, but they were practically horizontal in front. On the back they were sparse, and faintly developed in the interscapular region, He has consulted dermatologists and others, but nothing he has taken or used has done him any good. I only saw him once. Case 2.—Mr. H——, aged 37 years, manager of a factory, came to me on April 2nd, 1902. His general health was very good. The disease had been present five years, and began on the right fore-arm and a little later attacked the left. He has never been free since it first appeared, but thinks some patches have faded and others come out. In the last winter he had been decidedly worse, for the patches had Case 3.—Mr. H——, aged 56 years, butcher, came to me with skin lesions of which he had only been aware three weeks, but they may He was given salicin internally and, to rub in, 10 per cent. vasogen iodine. On February 11th there was decidedly less thickening on the left side but no noticeable difference on the right. He complained of indigestion, so the salicin was stopped and bicarbonate of soda given him, with tincture of nux vomica. On March 17th the thickening was much less and the patch on the left forearm was gone. On May 8th it had all cleared up and only left slight staining. As he has not come again he has probably remained well. The resolution of the patches was in this case probably, to be chiefly attributed to the vasogen iodine. Case 4.—Mr. M——, aged 54 years, a gentleman in good circumstances, was brought to me on June 2nd, 1903, by Dr. Lovell. All his family were long-lived. His grandfather was killed by an accident aged 93 years, but his father died of cardiac disease, aged 67 years. The disease the patient suffered from commenced early in April, i.e. Salicin 15 grains three times a day, with 5 minims of tincture of nux vomica, was prescribed. On June 23rd I saw him again, and there was then distinctly less thickening on the thighs and legs and the eruption was somewhat less bright in some parts. The longitudinal patches over the scapula were still thickened, but there was less infiltration in the patches on the upper limbs and on the forearms they were yellower. In response to my inquiry Dr. Lovell wrote me on November 29th, 1904, that he saw the patient on July 19th, 1903, and the eruption appeared to be gradually fading away. He next saw him for a sore throat in January, 1904, and the patient told him that the skin affection had gradually left him. He had had no other treatment than taking the salicin which I prescribed. This case is the most satisfactory and rapid in its involution under treatment, as it was also the most rapid in its evolution, and came earlier under my observation than any of the others. Case 5.—Dr. D——, aged 53 years. In 1876, while a house-surgeon, he first noticed a patch on one calf; since then the patches have gradually increased in number, and some of them in size. Chrysarobin kills them down for a time, but they re-form in the same place. In his family history a sister died of Addison’s disease, and one daughter is slightly phthisical. His own health is good and he is well nourished. When seen by me on February 4th, 1904, there was a large Case 6.—Mr. H——, aged 37 years, a draper. The disease had been present two years. When seen on April 26th, 1904, he had numerous patches on the calves and front of the legs, some three or four inches across, with distinct thickening. They were round, well-defined, and when pinched up were hard as compared with the adjoining healthy skin. Besides these there were more recent superficial irregular patches symmetrically arranged on both thighs. In the interscapular region and in front under the breasts were slightly yellowish stains, but no other alteration of the skin could be seen or felt, the surface being quite smooth. There was occasionally some itching, but never severe. There was constipation present, but no other symptom of ill health. He was ordered fifteen grains of salicin three times a day, and to rub in ten per cent. vasogen iodine. He was seen again on June 1st, when there was less thickening in some of the patches, but no other change. Case 7.—Mr. D——, aged 34 years, was seen first on November 3rd, 1904, in consultation with Dr. Payne. He had had a chancre in 1896. He had a severe sore throat and a rash, which only lasted a short time. He was treated with mercury for eighteen months. The present eruption began from three to four years ago, and for the last twelve months he had been treated by Dr. Payne with the biniodide of mercury mixture, 1/16 grain three times a day, and also with mercurial inunctions, but without making any material effect upon the present lesions. When I saw him the trunk was only slightly affected. There were a few pale yellow patches below the nipples, elongated and nearly horizontal. On the back, there were one He was ordered to rub in vasogen iodine and take salicin 15 grains three times a day. I heard about two months later by letter that there was no material change, but he had not used the iodine local application. 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 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. 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 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 seborrhoea 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 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. Brocq 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 Seborrhoea 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 seborrhoea, 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. (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. (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 oedema. Corium.—The vessels were dilated, with some cellular infiltration about them. The collagen appeared to be normal, with the exception of slight oedema, 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 qu 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%. |