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THE BRITISH JOURNAL OF DERMATOLOGY.

APRIL, 1895.

A CASE OF DESTRUCTIVE OF THE SCALP IN A PATIENT AFFECTED WITH HEREDITARY .

BY 3. F. PAYNE, M.D., F.R.C.P., Physician to Xt. Thomas’s Hospital, and to the Hospital for Diseases of the Xkin.

THE following case is one of a somewhat rare disease, which was first described by Quinquaud as or epilatoria, but here also complicated with more diffuse . In this case the history unmistakably proved, as will be seen, that inherited syphilis was the exciting cause; but whether this is also true of other cases of the disease I do not undertake to say. Quinquaud exhibited a case of this disease to the weekly meeting of the physicians of the St. Louis Hospital on December 13th, 1888; and on that occasion I find that Pournier stated he had seen a similar case in B young girl, which was due to hereditary syphilis.* Before this, apparently, Quinquaud had communicated a paper to the “Bulletin de la Sociitte Medicale des Hopitaux” in 1888.t He had also placed a coloured model of the affection in the museum of the Hospital of St. Louis in 1887 (Catalogice iles Moulaps, No. 1293). The affection has since then been frequently discussed, especially in the French journals. It is mentioned in Dr. Radcliffe-Crocker’s and Mr. Malcolm Morris’s works on diseases of the .

* Monafsht$te fiiv prakf.Dermatologie, IX. 474,1889 ; from Annaleo de Dernza- tologie, 1889. t The original is not accessible to me, but an abstract is contained in the British Jowrmal of Dermatology, Vol. I. p. 80. VOL. VII. I 102 DESTRUCTIVE FOLLICULITIS OF THE SCALP IN A

Before it had received a distinctive name, however, I thidi most dermatologists must have recognized destructive folliculitis of the head, and probably regarded it as a sort of sycosis of the scalp. In fact, the analogy with sycosis of the beard is almost perfect. It is a suppuration of the whole hair-follicle, including, doubtless, the sebaceous gland. The distinctive feature is the destruction of the follicle and permanent atrophy of the skin which results. But this also is not unknown in ordinary sycosis, in which distinct bald and atrophic areas are sometimes produced. I have seen the same result in Tinea sycosis or Tinea barb%, when the could be clearly demonstrated. h1. Quinquaud attributes the disease to a special form of bacterium which he isolated, and by of which into animals, destruction of the hairs mas produced. This may have been the true explanation of his cases ; but, considering the present case and the one alluded to by Fournier, it is possible that more than one cause may give rise to the same destructive process. The bald patches resulting from this affection have been compared to Alopecia arttata, and it is thought that it has previously been confounded with the latter disease. In my case, however, the con- dition of the skin was more definitely atrophic. One observation subsequently noted was that in several instances two or three hairs were seen growing from one hair-sac. This has often been observed in sycosis of the beard, and it was even suggested by Hebra that the growth of two hairs in a confined space might be the cause of the inflammation.* But with our present knowledge of the causes of suppuration this cannot be admitted ; and, moreover, the condition is not sufficiently universal. It seems more probable that the inflain- mation in its earlier stages so stimulates the growth of a secondary hair which is present alongside of the primary one that it projects with the former till both are loosened by suppuration. I will only observe, by way of precaution, that this condition is entirely different from the Dermatitis papillaris capillitii or - . HISTORYOF THE CASE. Sophie M., aged 14, was admitted to the Hospital for Diseases of the Skin as an out-patient, January 20th, 1892. She was small for * Hebra, Hautkrank7~citen, 1860, Vol. I. p. 629. He refers to Langer’s researches on the mode of growth of secondary hairs. PATIENT AFFECTED WITH HEREDITARY SYPHILIS. 103 her age, pale, and very cachectic, with fair complexion and blue eyes. She complained of a disease of the scalp, which had lasted six or seven years ; and for which she had been treated for some time at the skin department of a general hospital. It consisted of a suppurative affection of the hair-bulbs, with some diffuse inflammation producing crusts and scales, like a chronic . A large number of the hair-bulbs were absolutely destroyed, producing a large oval area of baldness some inches long, with glossy atrophic skin all over the vertex, and evidently spreading to the rest of the scalp. The hairs on the sides and back of the head and close to the forehead were com- paratively unaffected, but many showed suppuration of the follicles. There were no broken hairs, so that Tinea or Favus could be definitely excluded, but in several instances two or even three hairs were grow- ing from one follicle. The diffuse inflammation was also apparently of a destructive character, and assisted in producing the atrophic bald patches, but there was no true ulceration. On the ears and cheeks was an inflammation more like ordinary eczema. There was also a chronic inflammation of the eyelids of both eyes, for which the patient had been under treatment for a considerable time at the Ophthalmic Hospital, Moorfields. There was also chronic inflamma- tion at the margin of one nostril. The lymph-glands of the neck, under the jaw, and at the sides of the cheeks, in front of the ear, were enlarged and hard, but none of them had suppurated. The skin of the rest of the body was healthy, and there was no sign of pulmonary disease. The diagnosis was evidently destructive folliculitis, complicated with diffuse inflammation, also suppurative and destructive, and with some ordinary, perhaps seborrhceic, eczema. The general condition clearly pointed to some constitutional affection, such as tubercle of the scrofulous type, or inherited syphilis. Of tubercle, though the general appearance of the child was what is generally called scrofulous, there was really no evidence. The enlarged glands had never softened or suppurated, notwithstanding the long duration of the disease, and there was no evidence of any visceral or arthritic . The evidence for hereditary syphilis was carefully examined. The I2 104 DESTRVCTIVE FOLLICULITIS OF TIIE SCALP IN A teeth were suggestive, though not absolutely diagnostic of this affec- tion. The upper central incisors were iioriiial ; the second incisors were narrow aiid conical ; tlie upper canines only remarkabie in being rather short. The lower incisors were short, stunted, and separated by wide gaps. There was no deafness ; the cornere were clear, and no scars could be detected round tlie month. Notwithstanding the absence of that affection of the central incisors on which Mr. Hutchinson lays so inuch stress, and of other confirinatory evidence, the general appearance seemed to me to indicate late hereditary syphilis ; and this was remarkably corroborated by subsequent evidence, and by the results of treatment. FamiZy Ili.story.--Patient was the third of ten children, all of ~vhom are still living; the eldest was said to be quite healthy; the second had had ulcers on the eyes, evidently ulcerative keratitis. The remaining seven \yere said to be healthy, but I never saw any of the other children. There was no history of . The father and mother were said to be healthy. However, some two or three months after the child was admitted (and when anti- syphilitic treatment had been already commenced), the father accom- panied the child to the hospital, aiicl a single glmce at him at once showed the true state of the case. He was a tall, powerful man, but showed on his forehead some formidable scars, evidently due to tertiary syphilis; aiid sonie similar bald scars on the head. It appeared he had been in the army and had had primary syphilis, for which, he says, he was not treated. He was free from the symptoms for some years, and then married ; but about nine years from the primary disease he had an affection of the scalp and forehead which left the scam above mentioned. He was treated and com- pletely recovered, having had no sign of disease since. But quite recently (January, 1895) he has applied at the hospital for patches on the head, which are evidently a recrudescence of his old complaint. It is a suppurating, encrusted syphilide of the scalp, evidently such as would produce scars like those which have already been noted. The glossy atrophic scars are, I may say, something like those on the daughter’s head, but there is, in the father’s case, no follicnlitis. It is noticeable that the (late given for the outbreali of tertiary symptoms in the father coincided broadly with the appearance of liereditarg syphilis in his family. PATIENT AFFECTED WITH HEREDITARY SYPHILIS. 105

He stated that he had these symptoms about 1875, and they lasted some months. The second child, with ulcerative keratitis, must have been born about 1876, and our patient mas born in 1877 ; so that these would be the only children born or begotten while the father was thus suffering. It may also be noted that the inherited disease in this patient (and probably in her sister) was distinctly of a tertiary character (as’usnal in late hereditary syphilis), and that there was no history of any infantile affection, though such may possibly have been overlooked. This seems to me n fact in favour of the belief that the character of the of hereditary syphilis is influenced by the stage of the original disease in the parent from whom the inheritance has descended, and also shows the greater chance of hereditary at a time when the disease is active in the parent than in intervals of latency. Treatment.-When the child was first seen, a dilute nitrate of ointment with creosote was applied locally, and phosphate of iron internally. Under this treatment, which was continued for one month, no improvement was noted either in the local disease or in the general condition. Being convinced of the fact of syphilitic inheritance, I then prescribed Iodide of Potassium, first in three-grain and afterwards in ten-grain doses. There was an immediate improve- ment both in the local and general condition, which was still more marked when mercury (Hydrarg. cum cretL gr. ij) was also given. Afterwards a mixture of Perchloride of Mercury, first alone, and then with Iodide of Potassium, was substituted. In brief, the effect of this definite anti-syphilitic treatment was unmistakable. First the general nutrition improved, the child became fatter, and the colour returned to her cheeks, than which there can be no clearer evidence of the benefit of mercurial treatment. Then the local lesions gradually disappeared, so that 011 June 29th it was noted that the greater part of the head was healed, but some follicular sup- puration and crusts remained. The lymphatic glands also much diminished in size. Notwithstanding interruptions in the treatment (the child lived more than thirty miles from London, and only came up about once a month), the improvement was 80 marked that in October it was noted, “ most of the scalp clear ; no suppuration ; only some scaly patches.” Relapses however occurred in the course of 106 DESTRUCTIVE FOLLICULITIS OF SCALP WITH IIEREDITABY SYPHILIS. the next year, and it was tFo years before the head could be pro- nounced perfectly healed. It ma,y be worth noting that no local treatment was of much avail. Most of the remedies tried, such as various mercurial ointments, coal tar solution, creolin, sulphur, boric acid, either did no good or aggravated the inflammation. That which was most useful was an ointment containing iodoform. These results are decidedly against the assuniption of a parasitic cause for the disease, and confirm the diagnosis of syphilis. Cowdition after Treatnzeiit.-The girl has now a good growth of hair on the front, sides, and back of the head, but the bald vertical patch is quite unchanged, the hair-bulbs being entirely destroyed. Her general appearance is entirely altered. There is little or nothing in the physiognomy to suggest hereditary syphilis, and she is a well- nourished healthy-looking girl.