the sarcoidosis of boeck

1
1341 Most important of all is the continued responsi- bility of the obstetrician-in the antenatal, intra- partum, and postnatal care of the childbearing woman-for the application of the advances of modern medicine in every specialty. In the majority of cases the special maternity hospital will allow him to discharge this duty ; the ancillary departments of the maternity unit within a general hospital will permit it in all. THE SARCOIDOSIS OF BOECK Boeck’s sarcoid is familiar to most physicians as an uncommon and bizarre skin disease ; but there is more and more reason to think of this skin lesion as merely one manifestation of a generalised morbid process. SCHAUMANN has been urging this broader view since 1914 and it is now generally accepted that the skin is affected in only about half the cases. Dermatological mani- festations other than those described by BoECx- first as multiple benign sarcoids and later as miliary lupoid-are now admitted as part of the syndrome, the most important being lupus pernio, angiolupoid, and erythrodermie sarcoidique. The other main localisations of sarcoidosis are infil- trations of the phalanges of the fingers and toes, producing a deformity resembling spina ventosa and the radiological picture known as Jüngling’s osteitis multiplex cystica vel cystoides tuberculosa ; lymphadenopathy and splenomegaly ; pulmonary lesions with an irregular miliary distribution often associated with enlarged mediastinal and hilar lymph nodes ; and iridocyclitis. Infiltrations may be found in almost any organ or tissue ; enlargement of parotid and lacrimal glands has often been reported and BOECK himself wrote of the frequency of catarrhal symptoms due to mucosal lesions in the upper respiratory tract. The histological characteristics of all the lesions are the same : they consist of a focal proliferation of histiocytes to form collections of epithelioid cells with an occasional histiocytic giant cell. There is usually no surrounding lymphocytic infiltration and caseation is not seen. The tubercle bacillus is not found in sections, and animal inoculations-with one or two dubious exceptions- have been consistently negative, though the morbid histological change is what was described by ZIEGLER as " hyperplastic tuberculosis " and what many pathologists still call " endothelial tubercle." Evidence that the lesions are due to mycobacterial infection is not forthcoming and the Mantoux test is often negative. There is a wrangle of long standing over the tuberculous origin of sarcoidosis which recalls the dispute about the aetiology of Hodgkin’s disease, and a recent paper has once again raised this question. A case of sarcoidosis occurring in the son of a formerly phthisical man is described ; this boy had enlarged lymph nodes in the neck for five years and afterwards also in the mediastinum ; biopsy showed the typical histology of sarcoidosis and the Mantoux test was negative. Later he developed overt pulmonary tuberculosis with a positive intradermal reaction- 1 With, T. K., and Helweg-Larsen, P., Acta med. scand. 1938, 95, 92. contact with a case of open tuberculosis was elicited-and he finally died from miliary dissemina- tion. The authors show laudable caution and draw only the tentative conclusion that tuberculosis and sarcoidosis are in some way related. Reports of cases of this type are numerous and such obser- vations have often been employed as an argument favouring the tuberculous aetiology of sarcoidosis. The fallacies in this reasoning need no demonstra- tion and it is reasonable to regard the pulmonary tuberculosis as a secondary infection such as is acknowledged to take place in Hodgkin’s disease and leucosis. Any individual case of sarcoidosis may show lesions in one or more of the typical sites when first seen, but if it is kept under observation for a long time further lesions will appear and the earlier ones may retrogress. Symptoms of general intoxication, such as fever, loss of weight, and malaise, are often lacking and the course is extremely chronic with a tendency to spontaneous retrogres- sion and recoverv. It will be noted from the description we have given that if iridocyclitis and parotid gland enlargement are associated in the same case the symptom-complex known as uveo- parotitis or febris uveoparotidea of Heerfordt will be produced. Lymphadenopathy and spleno- megaly have often been described as occurring in this syndrome and the parotid gland shows the typical histological picture of sarcoidosis. Indeed recent reports 2 go far towards proving that uveoparotitis is no more than a clinical variant of sarcoidosis. It is interesting to reflect that it has required forty years of investigation to reveal the true nature of the latter disease. Even though its causation is still unknown it must now stand alongside Hodgkin’s disease and leucosis as one of the systematised proliferations of the lympho- reticular tissue-in the jargon of morbid histology, a histiocytic sinus reticulosis. IT would be ungrateful to allow Prof. SIGMUND FREUD to make our country his future home without bidding him welcome. His teachings have in their time aroused controversy more acute and antagonism more bitter than any since the days of Darwin. Now, in his old age, there are few psychologists of any school who do not admit their debt to him. Some of the conceptions he formulated clearly for the first time have crept into current philosophy against the stream of wilful incredulity which he himself recognised as man’s natural reaction to unbearable truth. Others have been discarded by all but a loyal band of psycho-analysts, and still others have been developed, extended, and altered so much as to make them hardly recognisable. FREUD comes to us at a time when one at least of his classic works is available to anyone who has 6d. to spend, and the audience to which he thus speaks directly is limitless. This is not the time to appraise his contribution, but to greet him in the hope that he may find peace and some joy among his many friends and admirers in London. 2 Longcope, W. T., and Pierson, J. W., Bull. Johns Hopk. Hosp. 1937, 60, 223 ; Slot, W. J. B., Goedbloed, J., and Gosling, J., Acta med. scand. 1938, 94, 74.

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Page 1: THE SARCOIDOSIS OF BOECK

1341

Most important of all is the continued responsi-bility of the obstetrician-in the antenatal, intra-partum, and postnatal care of the childbearingwoman-for the application of the advances ofmodern medicine in every specialty. In the

majority of cases the special maternity hospitalwill allow him to discharge this duty ; the ancillarydepartments of the maternity unit within a generalhospital will permit it in all.

THE SARCOIDOSIS OF BOECK

Boeck’s sarcoid is familiar to most physiciansas an uncommon and bizarre skin disease ; butthere is more and more reason to think of thisskin lesion as merely one manifestation of a

generalised morbid process. SCHAUMANN has been

urging this broader view since 1914 and it is nowgenerally accepted that the skin is affected in

only about half the cases. Dermatological mani-festations other than those described by BoECx-first as multiple benign sarcoids and later as

miliary lupoid-are now admitted as part of thesyndrome, the most important being lupus pernio,angiolupoid, and erythrodermie sarcoidique. Theother main localisations of sarcoidosis are infil-trations of the phalanges of the fingers and toes,producing a deformity resembling spina ventosaand the radiological picture known as Jüngling’sosteitis multiplex cystica vel cystoides tuberculosa ;lymphadenopathy and splenomegaly ; pulmonarylesions with an irregular miliary distribution oftenassociated with enlarged mediastinal and hilar

lymph nodes ; and iridocyclitis. Infiltrations maybe found in almost any organ or tissue ; enlargementof parotid and lacrimal glands has often been

reported and BOECK himself wrote of the frequencyof catarrhal symptoms due to mucosal lesions inthe upper respiratory tract.The histological characteristics of all the lesions

are the same : they consist of a focal proliferationof histiocytes to form collections of epithelioidcells with an occasional histiocytic giant cell.There is usually no surrounding lymphocyticinfiltration and caseation is not seen. The tuberclebacillus is not found in sections, and animalinoculations-with one or two dubious exceptions-have been consistently negative, though the morbidhistological change is what was described byZIEGLER as " hyperplastic tuberculosis

" and what

many pathologists still call " endothelial tubercle."Evidence that the lesions are due to mycobacterialinfection is not forthcoming and the Mantouxtest is often negative. There is a wrangle of longstanding over the tuberculous origin of sarcoidosiswhich recalls the dispute about the aetiology ofHodgkin’s disease, and a recent paper has onceagain raised this question. A case of sarcoidosisoccurring in the son of a formerly phthisical manis described ; this boy had enlarged lymph nodesin the neck for five years and afterwards also inthe mediastinum ; biopsy showed the typicalhistology of sarcoidosis and the Mantoux test wasnegative. Later he developed overt pulmonarytuberculosis with a positive intradermal reaction-

1 With, T. K., and Helweg-Larsen, P., Acta med. scand. 1938,95, 92.

contact with a case of open tuberculosis waselicited-and he finally died from miliary dissemina-tion. The authors show laudable caution anddraw only the tentative conclusion that tuberculosisand sarcoidosis are in some way related. Reportsof cases of this type are numerous and such obser-vations have often been employed as an argumentfavouring the tuberculous aetiology of sarcoidosis.The fallacies in this reasoning need no demonstra-tion and it is reasonable to regard the pulmonarytuberculosis as a secondary infection such as is

acknowledged to take place in Hodgkin’s diseaseand leucosis.

Any individual case of sarcoidosis may showlesions in one or more of the typical sites whenfirst seen, but if it is kept under observation fora long time further lesions will appear and theearlier ones may retrogress. Symptoms of generalintoxication, such as fever, loss of weight, andmalaise, are often lacking and the course is extremelychronic with a tendency to spontaneous retrogres-sion and recoverv. It will be noted from the

description we have given that if iridocyclitis andparotid gland enlargement are associated in thesame case the symptom-complex known as uveo-parotitis or febris uveoparotidea of Heerfordt willbe produced. Lymphadenopathy and spleno-megaly have often been described as occurring inthis syndrome and the parotid gland shows thetypical histological picture of sarcoidosis. Indeedrecent reports 2 go far towards proving that

uveoparotitis is no more than a clinical variantof sarcoidosis. It is interesting to reflect that ithas required forty years of investigation to revealthe true nature of the latter disease. Even thoughits causation is still unknown it must now standalongside Hodgkin’s disease and leucosis as one ofthe systematised proliferations of the lympho-reticular tissue-in the jargon of morbid histology,a histiocytic sinus reticulosis.

IT would be ungrateful to allow Prof. SIGMUNDFREUD to make our country his future home withoutbidding him welcome. His teachings have in theirtime aroused controversy more acute and antagonismmore bitter than any since the days of Darwin.

Now, in his old age, there are few psychologists ofany school who do not admit their debt to him.Some of the conceptions he formulated clearly forthe first time have crept into current philosophyagainst the stream of wilful incredulity which hehimself recognised as man’s natural reaction to

unbearable truth. Others have been discarded byall but a loyal band of psycho-analysts, and stillothers have been developed, extended, and alteredso much as to make them hardly recognisable. FREUDcomes to us at a time when one at least of his classicworks is available to anyone who has 6d. to spend,and the audience to which he thus speaks directlyis limitless. This is not the time to appraise hiscontribution, but to greet him in the hope that hemay find peace and some joy among his many friendsand admirers in London.

2 Longcope, W. T., and Pierson, J. W., Bull. Johns Hopk.Hosp. 1937, 60, 223 ; Slot, W. J. B., Goedbloed, J., and Gosling,J., Acta med. scand. 1938, 94, 74.