204

SARCOID OF BOECK" REPORT OF A CASE.

By DAvm M~TCHELL.

A LTHOUGH this interesting, if rather rare, condition has

attracted much attention in recent years, particularly in American and Scandinavian literature, there appears

to have been no case so far recorded in Ireland. Before describing the present case, a brief review of the conno-

tation of the t e r m " Boeck's sarcoid " may be introduced. The sub- ject has been fully covered in papers in English by Longcope and Pierson (1937) and by Snapper (1938), and the following summary cf the subject is largely taken from their complete accounts.

Boeck's sarcoid may be defined as an infective granuloma of un- known origin, histologically resembling tuberculosis more closely than any of the other recognised diseases of this type (syphilis, leprosy, actinomycosis, etc.). The granulomata have a charac- teristic distribution in the body affecting the lymph nodes, both superficial and those at the lung roots, the skin, the small bones of the hands and feet, and certain parts of the eye. The sarcoid tissue has recognised histological characters. It occurs in well- defined " hard " tubercles, that is to say, more or less spherical groups of epithelioid cells with a few giant cells of the Langhans type, with relatively few surrounding lymphocytes, and, most important, with no tendency to necrosis and caseation. There is little connective tissue reaction in the surrounding normal tissue. Although this sarcoid tissue is the same at every site, the effects produced are varied. For example, in the skin where the nodules are in the cutis, the external appearances vary from numerous small discoloured, even warty, papules to the diffuse bluish-red dis- coloration and swelling of lupus pernio. In the bones, the sarcoid tissue develops in the marrow and causes decalcification, showing varying patterns, most often of circular punched-out areas. The same process in the superficial lymph nodes produces moderate painless enlargement of those affected. The glands are firm, mobile and discrete, resembling those of Hodgkin's disease. In the lungs, the enlargement of the root glands may cause symptoms and in some cases there is a dissemination of nodules of sarcoid tissue of miliary size through both lungs. Again, in the eye, nodules of sarcoid tissue may be found in the iris and ciliary body or, more rarely, in other parts of the eye as in the present case.

Clinically, the disease begins in early adult life and runs a pro- longed and benign course with a tendency towards spontaneous healing. 'Constitutional disturbances or local symptoms are notably rare, even when the pathological changes are extensive; the most frequent feature is the enlargement of at least a few superficial lymph glands, of which those in the neck are most commonly affected; they are smooth, firm, movable and not tender; the enlargement is never very great. Skin lesion~g occur in about 50 per cent. of the cases, and pulmonary involvement to about the same extent. The

SARCOID OF BOECK 205

latter may cause difficulties in diagnosis as the enlarged root glands may cause cough and dyspncea and the disseminated nodules in the lungs may be mistaken for miliary tuberculosis or silicosis.

The etiology is quite obscure. The histology of the lesions sug- gests tuberculosis, but this has never been satisfactorily shown to be the causative agent; the tuberculin reaction is usually negative and acid-fast bacilli have rarely, if ever, been demonstrated in the lesions of the skin or lymph glands. Similarly, animal inocula- tions from these tissues are almost always negative for tuberculosis.

CASE REPORT.----W. F . , a ma le shop ass i s t an t , aged 28 years , was a d m i t t e d to t h e Adela ide Hospi ta l on Sep tember 29th , 1941, compla in ing o f sk in t r oub l e on b o t h t h u m b s a n d t he r ight index finger. Hi s prev ious h i s to ry was nega t ive . His work involved selling over a coun te r in a c o u n t r y general s tore. He d a t e d his p re sen t i l lness f rom J u n e , 1940, when t he t op o f t he r igh t i ndex finger became swollen a n d painful . Six m o n t h s later , in December , 1940, he began to no t i ce t h a t b o t h t h u m b s were swollen. A t different t i mes before admiss ion he h a d been t r ea t ed elsewhere, b y enclosing t he fingers in p las te r o f Par i s for several m o n t h s , b y local u l t ra-viole t rad ia t ion f rom a K r o m a y e r l amp , a n d later , b y f rac t ional doses o f x - rays ; these t r e a t m e n t s h a d l i t t le effect on t h e condi t ion of his fingers. The p a t i e n t t h o u g h t t h a t t he u lcera t ion p resen t on t h e r igh t i ndex finger a n d r ight t h u m b began a b o u t two weeks af ter t he x - r ay t r e a t m e n t ceased, p e r h a p s four or five m o n t h s before admiss ion . There was pract ica l ly no pa in a t t he t i m e of e x a m i n a t i o n , a n d he h a d no o ther s y m p t o m s .

On e x a m i n a t i o n the pa t i en t was found to be well developed, b u t r a the r t h i n , w i th a t h i n sk in a nd pecul iar ly b r igh t red complexion. His pulse, t empe ra tu r e a n d respi ra t ions were n o r m a l ; he was myop i c a n d wore glasses. A careful cl inical e x a m i n a t i o n showed no abnormal i t i es except in t h e eyes, left ear , b o t h handB and t h e r ight foot. T he left superficial cubi ta l l y m p h g land was enlarged to t h e size o f a smal l bean , was f i rm, freely movab l e a n d n o t tender . No other superficial g lands were felt.

On t h e ant i -hel ix o f t he left ear was seen a n a rea a b o u t 1 cm. in d i amete r , which was red, s l ight ly raised a n d scaly. T h e t e rmina l pha l anges o f bo th t h u m b s a n d of t he r ight index finger were enlarged a n d d is tor ted p rox ima l to t h e na i l fold ; on t he r ight t h u m b the re was a shal low ulcer, a b o u t 1 cm. in d i amete r , t he floor o f which was covered wi th pale , r a the r d ry g r anu l a t i on t issue. X - r a y e x a m i n a t i o n showed a h igh degree o f decalcification o f t he af fected pha l anges , t h e second p h a l a n x of t he r igh t index showed similar changes to a less degree. The t h i rd toe o f the r igh t foot showed a painless fus i fo rm e n l a r g e m e n t ; t h e r ad iog ram of th i s showed no bone lesion. The chest , which was n o r m a l on clinical e x a m i n a t i o n , was x- rayed, b u t showed no a b n o r m a l i t y excep t for we l l -marked hi lar g lands .

The resu l t s o f oph tha lmic e x a m i n a t i o n p roved to be o f g r ea t in teres t , not only because t h e y s t rong ly s u p p o r t e d t he diagnosis b u t because t he local isat ion o f t he nodu les was in t he re t ina , where t h e y h a v e n o t c o m m o n l y been repor t ed in Boeek ' s sarcoid. The following is Dr. Maxwel l ' s repor t : - -

" T h e pa t i en t , when seen in October , 1941, was wear ing R . E . - - 1 spher ical L .E . - - 7 spherical.

" A p a r t f rom th is m y o p i a he h a d n o t exper ienced h i the r to a n y difficulty in vis ion, b u t since t he inves t iga t ion wh ich revealed to h i m a n ocular defect , he h a s no t i ced t h a t ' t he re is t he image o f t he figure 7 over t h e r igh t eye m~l~ing the t o p s o f objec ts ind is t inc t . '

The f indings on e x a m i n a t i o n were as follows : - -

R .E . - - 1.5 sph. ~ - ~ 1 J . 4. L .E. - - 7 sph. 8 - - 5 J . 1.

" A screen tes t revealed in t h e r igh t eye a well-defined pa racen t ra l s c o t o m a above a n d to t he nasa l side o f t h e b l ind spo t which l a t t e r showed en l a rgemen t on i ts n a s a l aspect .

" Oph tha lmoscop ic e x a m i n a t i o n o f the right eye showed severa l e leva ted grey ish masse s over ly ing t h e re t ina l b lood vessels. T h e y were g rouped m a i n l y a long t h e t empora l border o f t h e opt ic nerve . T h e larges t m e a s u r e d roughly t disc d iameter . No a b n o r m a l i t y was discovered in t h e left eye.

206 IRISH JOURNAL OF MEDICAL SCIENCE

" From an ophthalmological viewpoint this case is of especial interest as the literature suggests that fundns lesions in this disease are of rare occurrence."

A biopsy section was taken from the ulcerated area on the right thumb. The histological report was that there were round defined groups of epithelioid cells, with one or two giant cells and some lymphoeytes a t the periphery. There was no trace of caseation or inflammatory reaction in the surrounding eutis. The left superficial eubital lymph gland was removed and one half was used for guinea. pig inoculation, the other half being sectioned, this presented an identical his. tological appearance with tha t of the skin section. Sections from both skin and gland were stained by Ziehl Neelsen's method, but no acid-fast bacilli were found. The guinea-pig which had been injected with material from the lymph gland was killed after seven weeks and showed no signs of tuberculosis.

The Wa~sern~nn reaction was negative. Tuberculin testing was carried out both by the Volmer patch test and by the intracutaneous injection of P.P.D. with entirely negative results.

Since the above account was written we have had an opportunity (April, 1942) to e~arnlue this pat ient again. The condition of the fingers and toe was practically unchanged. The latter still showed no radiological change. The pat ient 's general condition remained good. On the right cheek over the malar bone there was found a patch of diffuse thickening in the skin of about 3 cm. diameter. The skin surface appeared unchanged. The most striking difference was found in the condition of the right eye, which had deteriorated considerably. The greyish masses previously seen on the temporal side of the optic nerve had coalesced and now appeared as yellowish white plaques. The vitreous humour had become hazy, which accounted for the reduction of vision, which amounted to the counting of fingers close to the eye. The left eye remained as before.

Summary. The diagnosis of Boeek's sarcoid was made from the typical his-

tdogical appearance of the tissue from the skin and lymph node. This was supported by the negative tuberculin tests and by the x-ray findings. Clinically, the patient's age, the absence of con- stitutional symptoms and the prolonged benign course were characteristic. Small cellular nodules were found in relation to the retinal blood vessels in one eye. Ulceration of the skin lesions and the localisation of the eye lesions in the retina rather than in the iris and ciliary body were unusual features.

ROYAL COLLEGE OF SURGEONS IN IRELAND.

CARMICHAEL PRIZE ESSAY.

The President and Council give notice that on the second Thursday in June, 1943, they will proceed to adjudge prizes of One Hundred and Fifty pounds and One Hundred pounds for the best essays submitted on or before 1st February, 1943, on " The State of the Medical Profession in Great Britain and Ireland, etc. ," in accordance with the instructions prescribed by Mr. Carmichael.

Further particulars may be obtained from the Registrar, Royal College of Surgeons in Ireland, St. Stephen's Green, Dublin.