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J Clin Pathol: first published as 10.1136/jcp.38.7.797 on 1 July 1985. Downloaded from J Clin Pathol 1985;38:797-800

Isolated arteritis of the epididymis

C WOMACK, ID ANSELL From the Departments ofHistopathology, University and City Hospitals, Nottingham

SUMMARY The clinical, histological, and immunohistochemical features of two cases of appar- ently isolated arteritis of the epididymis are presented. The aetiology and pathogenesis of the condition are discussed. Immunoglobulin and complement were shown in the acute arterial lesions, but this is not conclusive evidence that isolated arteritis is either an disease or a forme fruste of polyarteritis nodosa.

Necrotising arteritis apparently confined to one CASE 2 organ yet showing histological features similar to A 14 year old boy was noted to have an unde- those seen in polyarteritis nodosa is now well recog- scended right testicle at a routine school medical nised. Since the first description of isolated arteritis examination. There was no history of trauma, there in the appendix by Plaut' similar apparently isolated were no urogenital symptoms, and the patient was arteritis has been recorded in the gall bladder,2 otherwise fit and well. On examination the right tes- uterine cervix,34 uterine body,5 skin,6 and breast.' ticle was in the inguinal canal and was small and The British testicular tumour panel material8 non-mobile. The left testicle was clinically normal. included three cases of necrotising arteritis; in one The patient underwent a right orchidectomy patient the disease was present only in the testis, but because the cord was insufficient to perform copyright. his subsequent clinical history is unknown. We orchidopexy. He made an uneventful postoperative report two cases of apparently isolated arteritis of recovery and is in good physical health six months the epididymis in patients who have no clinical evi- later. The resected testis measured 3-5 x 2-0 x 2 0 dence of systemic disease. cm. The testis, epididymis, and cord appeared nor- mal macroscopically. Case reports Both patients were normotensive and neither was http://jcp.bmj.com/ taking any drugs before presentation. CASE 1 A 37 year old man presented with a tender mass in INVESTIGATIONS the left testicle which had appeared suddenly three Table 1 shows the results of relevant postoperative weeks previously. There was no history of recent haematological and biochemical investigations. All trauma. An injury to the same testicle some 20 years were within the normal ranges of the laboratory.

before had preceded a similar tender mass, which Blood eosinophil counts were not performed. on September 26, 2021 by guest. Protected had disappeared spontaneously. There were no other urogenital symptoms and the patient was HISTOLOGY otherwise fit and well. On examination there was a Routine paraffin embedded tissue sections roughly hard, irregular, tender nodule at the lower pole of 7 ,um thick were stained by haematoxylin and eosin, the left epididymis; the right testicle was clinically Martius scarlet blue, and elastic Van Gieson. In normal. The patient underwent a left orchidectomy case 1 the testis showed no appreciable histological via an inguinal incision. He made an uneventful abnormality; in case 2 there was complete absence postoperative recovery and is in good physical of germ cells consistent with maldescent. There was health two and a half years later. The resected testis a multifocal necrotising arteritis affecting the measured 6 5 x 3-5 x 3-0 cm and showed no mac- medium and small muscular arteries of the roscopic abnormality. The lower pole of the epididymis in both cases and of the spermatic cord in epididymis contained a well defined, hard white case 2. The acute arteritic lesions were characterised mass 2 cm in diameter. by focal of the vessel wall with an associated infiltrate of neutrophil polymorphonu- clear leucocytes and lymphocytes (Fig. 1). Accepted for publication 14 March 1985 Eosinophil polymorphonuclear leucocytes were not 797 J Clin Pathol: first published as 10.1136/jcp.38.7.797 on 1 July 1985. Downloaded from 798 Womack, Ansell Table 1 Haematological and biochemical results Case Blood Serum Erythrocyte sedimentation Haemoglobin White cell count Urea concentration Creatinine concentration rate (mm in the first hour) concentration (gldl) (X109/1) (mmolNl) (pinolll) 1 7 13-8 5-7 4-3 82 2 1 15-0 5-6 4-5 87 Values are all within the laboratory normal ranges.

seen and the fibrinoid material stained red with Mar- Discussion tius scarlet blue. In some arteries the acute lesions were circumferential. Some arterial lesions were The diagnosis of isolated arteritis depends on the characterised by pronounced intimal (fibrous) thick- clinical exclusion of systemic disease in a patient ening and an infiltrate of lymphocytes in the muscu- who has histological evidence of a necrotising lar wall without fibrinoid necrosis (Fig. 2). Around arteritis apparently limited to one organ. The his- all the abnormal arteries there was a lymphocytic tological picture is similar to that seen in classic infiltrate of variable intensity, which was most strik- polyarteritis nodosa, although there are some minor ing around the lesions showing fibrinoid necrosis. differences in cases of isolated arteritis, notably the lack of thrombosis, aneurysm formation, or infarc- IMMUNOHISTOCHEMISTRY tion.3 In some reports the lesions are described as Paraffin sections were stained for IgG, IgM, IgD, granulomatous,7 9 "' but the presence of multinucle- IgA, hepatitis B, and complement components Clq ated giant cells close to disrupted fragments of arter- and C3 using a standard peroxidase-antiperoxidase ial elastic lamina (a feature not specific to temporal was by Dako UK Ltd. technique. Antisera supplied arteritis) may occur secondarily to vessel wall dam-copyright. Positive staining was only seen in acute arteritic age." lesions (Table 2). To our knowledge isolated arteritis of the http://jcp.bmj.com/ on September 26, 2021 by guest. Protected

Fig. 1 An acute arteritic lesion. Necrosis and fibrinoid change affects part ofthe circumference ofthis artery. There is a moderate inflammatory cell infitrate around the necrotic area and in a perivascular distribution (from case 1). Haematoxylin and eosin. Original magnification x135. J Clin Pathol: first published as 10.1136/jcp.38.7.797 on 1 July 1985. Downloaded from Isolated arteritis of the epididymis 799

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not reported. Both regularly exposed to a wide variety of antigenic epididymis has been previously copyright. of our patients had evidence of necrotising arteritis material and an Arthus type reaction can reasonably of the epididymis without clinical evidence of sys- be proposed as a cause for isolated arteritis at these temic disease. The British testicular tumour panel8 sites. Similarly, the breasts may be exposed to new include three cases of necrotising arteritis: one during pregnancy and lactation, although patient later developed renal lesions and another the recorded cases of isolated arteritis in this organ had . The third patient in the have occurred in middle aged and elderly women.7 panel series had a of the testis, but Potentially antigenic material may be localised in http://jcp.bmj.com/ his subsequent history is unknown. the epididymis following trauma or retrograde flow The aetiology of isolated arteritis is unknown. of urine down the vas deferens.8 In our two cases Necrotising arteritis is well recognised as a complica- there was no history of recent trauma that could tion of irradiation, but in our cases and in other explain the acute arterial lesions. Neither patient reports of isolated arteritis there was no history of complained of urinary symptoms, but we do not previous irradiation. Many forms of arteritis are know whether there was retrograde flow of urine

considered to be mediated by immunological down the vasa, the likely mechanism of localisation on September 26, 2021 by guest. Protected mechanisms. Isolated arteritis may represent a local- of to the epididymis if a type III hyper- ised type III (Arthus) reaction." sensitivity reaction is entailed in the pathogenesis of Such hypersensitivity to an inhaled antigen has been the arterial lesions. The immunohistochemical suggested as one of the causes of the necrotising staining of our two cases is not, however, conclusive , Wegener' s granulomatosis, although no evidence of an immune complex mediated disease. antigen has yet been identified." Sites such as skin, The presence of immunoglobulin and complement appendix, cervix, uterus, and gall bladder are in acute arteritic lesions may be due simply to

Table 2 Immunohistological staining ofacute arteritic lesions Case lgG IgM IgA IgD Clq C3 Hepatitis B 1 + + - - + - - 2 - + - + + = staining in acute artentic lesions. - = no staining in acute arteritic lesions. J Clin Pathol: first published as 10.1136/jcp.38.7.797 on 1 July 1985. Downloaded from 800 Womack, Ansell passive diffusion of plasma constituents through References arterial walls damaged by another mechanism. Plaut A. Focal arteriolitis. Am J Pathol 1932; 8:620-1. We have already noted the histological similarities 2 Bohrod MG, Bodon GR. Isolated polyarteritis of the gall blad- between the lesions of isolated arteritis and polyar- der. Am Surg 1970; 36:681-5. teritis nodosa. Polyarteritis nodosa is considered to -Ansell ID, Evans DJ, Wright DGD. Asymptomatic arteritis of the uterine cervix. J Clin Pathol 1974; 27:664-8. be an immune complex mediated disease, and 30% Crow J, McWhinney N. Isolated arteritis of the cervix uteri. Br J of patients with the disorder have circulating Obstet Gynaecol 1979;86:393-8. hepatitis B antigen.'2 We were unable to show Lin CS, Brazza F. Focal arteritis of uterus. A case report. Mt Sinai J Med 1978;45:402-3. hepatitis B antigen immunohistochemically. The 6 Cupps TR, Fauci AS. The vasculitides. In: Cupps TR, Fauci AS positive immunohistochemical staining for IgM and eds. Major problems in internal medicine, vol XXI. Philadel- C3 which we have shown in our cases of isolated phia: WB Saunders, 1981. arteritis is similar to the findings of other workers in Chiatin B, Kohout ND, Goldman RL. Focal arteritis of the acute arterial lesions of polyarteritis nodosa.'2 breast. Angiology 198 1; 32:334-7. the Morgan AD. Inflammation and infestation of the testis and para- This similarity may not have much significance since testicular structures. In: Pugh RCB, ed. Pathology ofthe testis. we have already noted that the finding of immuno- Oxford: Blackwell Scientific, 1976:119. globulin and complement in arteritic lesions does Pirozynski WJ. Giant-cell arteritis of the uterus. Report of two not prove an immune complex pathogenesis. cases. Am J Clin Pathol 1976;65:308-13. '° Lasser A, Ghofrany S. Necrotising granulomatous vasculitis Clinically, the two diseases are strikingly differ- (allergic granulomatosis) of the gall bladder. Gastroenterology ent. Polyarteritis nodosa, if untreated, has a poor 1976; 71:660-2. prognosis; death, usually resulting from renal failure Mitchinson MJ. The vasculitis syndromes. In: Anthony PP, or cardiovascular or intestinal complications.6 13 Iso- MacSween RNM, eds. Recent advances in histopathology, vol 12. Edinburgh: Churchill Livingstone, 1984:223-40. lated arteritis is often asymptomatic and has an 2 Heptinstall RH. Polyarteritis (periarteritis) nodosa, other forms excellent prognosis. of vasculitis and rheumatoid arthritis. In: Heptinstall RH ed. It is important that any patient with histological Pathology ofthe kidney, vol 11. 3rd ed. Boston: Little, Brown evidence of necrotising arteritis should have full and Co. 1983:793-838. clinical, haematological, and biochemical investiga- 'Fauci AS. Vasculitis. J Clin Immunol 1983;72:211-23. tions to exclude systemic disease because of the clini- copyright. cal differences between polyarteritis nodosa and iso- lated arteritis. We thank Mr AN Fawcett and Mr CP Bates for permission to publish clinical details of their

patients, Mr W Brackenbury for photographic assis- Requests for reprints to: Dr C Womack, Department of http://jcp.bmj.com/ tance, and Miss Flora Ottawa for typing the original Histopathology, University Hospital, Nottingham draft. NG7 2UH, England. on September 26, 2021 by guest. Protected