Part 1

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The British Journal of Dermatology, April, 1905

THE BRITISH JOURNAL OF DERMATOLOGY.

APRIL, 1905.

XANTHO-ERYTHRODERMIA PERSTANS. By H. RADCLIFFE-CROCKER.

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 intervals, large areas are involved, and in the course of years (in one case, months) the whole trunk and limbs are crowded with lesions, though there are always spaces of normal skin intervening, or sometimes completely enclosed by the diseased process, where the original patches have coalesced. For the most part the original patches are discrete and enlarge but little after their formation, unless they merge into adjoining patches, when hand-sized or larger areas may be formed.

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 then the trunk. The lower limbs, too, are generally more crowded with lesions than other parts.

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 in the other cases; while there was some spontaneous improvement in the summer, in winter the patches cracked and smarted. This patient, who had been affected with the disease for ten years, had had the most varied drug and spa treatment, including cacodylate of soda injections for three months without any material effect; but after nine exposures to the Röntgen rays, the part exposed entirely cleared up, while the disease was unchanged on the inner side of the leg which the rays had not reached. I intend, therefore, to make use of the rays wherever practicable.

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.

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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, and not nearly so numerous as in front; but on the lower half of the back and sides they were in the form of yellowish red stains, without elevation or roughness, and they were more numerous than on the upper part of the back. Forearms: The patches were on the inner side chiefly, more numerous on the right side than the left, and thickly arranged between the wrist and elbow. They were not elongated, but roundish, oval, or irregular, about three quarters of an inch in diameter, and somewhat brighter in tint than on the trunk. The surface was faintly rough, and on pinching up a patch it was slightly thicker than usual. There were about eight to ten patches on the right upper arm, while the left was almost free, and there were not nearly so many patches on the left forearm as there were on the right. On the thighs: They were most abundant on the inner side, and many more on the right side than on the left. There were scarcely any patches on the front of the thigh, but there were a few on the outer side. The patches sloped downwards and inwards, were oval and broader than the trunk patches, but still like finger-streaks. The legs were much more densely covered with coalesced irregular patches, some enclosing healthy skin, and the surface was rougher than the lesions on the rest of the body. On the neck, there were a few ill-defined spots about one inch square in area. There was slight blotchy redness of a not very obvious kind on the face, scarcely perceptible on the forehead. There was some seborrhœa capitis, but not so much as formerly, as he uses brilliantine. The patches seldom itched unless he got very hot. No illness preceded the eruption; in fact, he has never had any illness; his tongue was clean, and he looked and has always been perfectly healthy. The general aspect was somewhat that of a general orbicular seborrhoic eczema, except that for the most part the surface was smooth.

He has consulted dermatologists and others, but nothing he has taken or used has done him any good. I only saw him once.

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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 certainly increased during the last few months. On the fore-arms, they were nearly symmetrical, and were quite so at an earlier stage. The lesions were yellowish or pale red patches; the simple ones were elongated, but the compound ones irregular in outline; they were from 3/4 to 1-1/2 inches long. The surface, with a lens, could be seen to be very slightly roughened, but this was not perceptible to the finger—but when pinched up there was decided thickening, though they were not raised. There were no patches on the back of the arms or fore-arms and none on the trunk, but there were similar patches on the inside of the thighs and on the legs, some of them larger than the arm-lesions but less distinctly patchy. There was a large, irregular area on the back of each thigh, and a few roundish ones scattered about the limbs. The face was quite free, there was no seborrhœa capitis and there was no itching or other sensory symptoms in the patches. He was one of ten brothers and sisters who were in good health, except one who died of syphilitic paralysis. His father died aged 71 years, and his mother, aged 63 years, was alive and well. He was given salicin, gr. xv _ter die_, and a lotion of glycerine of lead and liquor carbonis detergens. I saw him again on July 3rd, when the eruption had cleared up considerably, much of it having faded, leaving only a slight stain, but here and there it was still yellow and slightly rough. He was much better when he took the medicine regularly, but when he stopped it some of the eruption returned. The salicin was increased to 20 grains. I did not see him again until July 23rd, 1904. He then said he took the medicine for three months from July 3rd, 1902, and then stopped it. He had used the lotion a little longer. On the back of the legs there were large patches nearly covering the entire area, but not much in front. It was more distinctly red here than elsewhere, and there was very little thickening. There were only one or two patches on the left thigh, but a dozen or more on the left. The trunk still remained free. The initial patch was on the right wrist seven and a half years previously, but had disappeared. Some few fresh ones still developed occasionally. He was told to continue the salicin and rub on 10 per cent. vasogen iodine.

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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 have been there longer as he had to get up so early to attend the markets that he rarely saw his own skin. He was a stout healthy-looking man, but he had syphilis in 1879 and had then suffered from iritis; he was under treatment for two years. He suffered from constipation and had done so since he had enteric fever as a young man. Urine had no albumen nor sugar. He came of a long-lived family, his father having died at 86 and his mother at 74. He had seven brothers and sisters alive out of thirteen. The lesions were few in number and situated symmetrically over the lower ribs on each side, the patches sloping obliquely downwards from the back to the front. One on each side was 6 or 7 inches long and compound. There was a single one much smaller (2-3 inches) symmetrically situated in a line with the large patch, and there were three or four others above the major patch on the back. There was one distinct patch over the left scapula and about half a dozen fainter ones about the back; a few faint ones were present on the back of each arm and forearm. The lower limbs were free. The patches were erythematous in aspect, the larger ones brightish red, the others paler. The surface was very faintly roughened to the touch, and there was very distinct thickening when the skin was pinched up, but they were not raised above the surface. The borders of some of the patches were well defined, while others shaded off gradually.

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.

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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._ two months before I saw him, on the right leg, and by June had extended all over the lower limbs and on the trunk, nearly all over the back, but there was scarcely any on the front. On the forearms, the patches were more on the extensor than the flexor surface. The eruption consisted of irregular, pale, erythematous patches from a square inch to the palm in size, with distinct infiltration in most of them. On the back, they were arranged symmetrically in the direction of the ribs, _i.e._ sloping downwards and forwards from back to front. In the early stage, on the back, they could be traced to commence as minute papules at the hair-follicles, and gradually the intervals between were filled up until infiltrations with a uniform surface were formed. Some itched, but none severely, and many did not itch at all.

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.

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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 irregular lesion occupying nearly the whole of the interscapular region and numerous others on the back, abdomen, and thighs. They were of a dull pale lemon colour; some were distinctly thickened when the skin was pinched up; in others this was not recognisable. On the thighs, they were pinkish and slightly roughened. On the body they were smooth, elongated, oblong, oval, and well defined, symmetrically placed, and on the back in oblique lines sloping down and out from the spine in the lines of cleavage, _i.e._ corresponding to the rib slope. They seldom itched or gave any trouble.

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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.

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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 or two commencing yellowish spots in the interscapular region, but in the lumbar and sacral regions they were fairly numerous, and also yellowish and elongated horizontally. On the thighs, the patches were very abundant in vertical elongated streaks about half an inch wide, as if the finger had been wiped down the limb in two- or three-inch pale yellow streaks. On the legs, the patches were larger, irregular in outline from coalescence, and of a yellowish-pink colour. The eruption was very symmetrical, many of them, especially on the thighs, were slightly but distinctly thickened when pinched up. On the anus and forearms there were a good many patches, but not so many as on the thighs, where, arranged in the line of the limb, they were of a paler yellow colour. The patient said that they itched very decidedly almost always at night and very often in the day. Sometimes they were sore. They did not itch when they first came out. The eruption began on the thighs, and although fresh ones were coming out from time to time, none, so far as he knew, had faded or gone away.

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.

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