THE JOURNAL OF UROLOGY

Vol. 115, March Printed in U.S.A.

Copyright© 1976 by The Williams & Wilkins Co.

SCROTAL SWELLING IN THE SCHONLEIN-HENOCH SYNDROME VERNA JEAN TURKISH, HOWARDS. TRAISMAN, A. BARRY BELMAN, GILBERT Z. GIVEN THOMAS J. MARR*

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From the Department of Pediatrics and the Division of Urology, Children's Memorial Hospital and the Departments of Pediatrics and Urology, Northwestern University Medical School, Chicago, Illinois

ABSTRACT

Testicular and scrotal hemorrhage is uncommon in the Schonlein-Henoch syndrome. The diagnosis of testicular torsion is difficult to make when it is complicated by vasculitis of the testis. Since both conditions can occur simultaneously, torsion of the testis must be considered and surgical exploration performed whenever acute testicular pain and swelling occur during the course of the Schonlein-Henoch syndrome. Two patients with this syndrome as well as scrotal and testicular involvement are described. The Schonlein-Henoch syndrome or anaphylactoid purpura is a disease of unknown etiology that is characterized pathologically by a diffuse acute vasculitis of the small blood vessels. The disease is relatively common in children and several reviews of large series of patients have been reported.'· 2 The classical picture of the Schonlein-Henoch syndrome consists of a characteristic rash, joint symptoms, abdominal pain, edema, gastrointestinal bleeding with occasional perforation and intussusception, and sometimes renal abnormalities similar to those seen in post-streptococcal glomerulonephritis. 2 • 3 The rash may be urticaria! in appearance, with red maculopapular lesions that appear initially and gradually fade to reddish-brown patches, or it may be petechial or ecchymotic. Its classic distribution over the buttocks, perineum and lower extremities is most helpful in establishing the diagnosis. The platelet count is normal or elevated. Rare instances of neurologic involvement have been reported with convulsions, facial nerve palsy, hemiparesis and coma.•-• Roy described a case of Schonlein-Henoch syndrome complicated by steatorrhea and malabsorption. • The disorder may occur without the characteristic purpuric rash or with manifestations limited initially to internal organs and the appearance of a rash sometime after the onset of symptoms. In rare cases the rash may never occur. 7 Reference to involvement of the male genitalia was first made in 1960 by Allen who described testicular and scrotal hemorrhage in this syndrome. 1 In 1968 Fitzsimmons reported testicular pain and scrotal swelling that was severe enough to simulate acute torsion of the testis. 4 Herein we report on 2 patients with scrotal and testicular involvement who were recently seen at the Children's Memorial Hospital. CASE REPORTS

Case 1. A 7-year-old white boy had a purpuric rash over the pretibial areas, pain in several of the large joints, abdominal pain and vomiting 1 week in duration. He had had an upper respiratory infection 2 weeks before hospitalization associated with fever and a sore throat. Because the abdominal pain worsened and the patient began to pass grossly bloody stools, he was admitted to another hospital and started on 5 mg. prednisone 3 times daily. The rash as well as joint and abdominal pains disappeared during the next 3 days, and the patient was discharged from the hospital. Later that day he again had purpuric spots on the legs, periumbilical abdominal pain and a tender, swollen, erythematous scrotum. The patient was admitted to Children's Memorial Hospital, complaining of modAccepted for publication July 3, 1975. * Requests for reprints: 1325 W. Howard St., Evanston, Illinois 60202.

erately severe abdominal pain. Temperature was 37.8C orally, pulse 100 and blood pressure 100/70. The abdomen was soft with normal peristaltic sounds and slightly tender below the umbilicus. Numerous purpuric lesions were noted over the pretibial areas and buttocks (fig. 1). Marked swelling, tenderness and erythema of the left side of the scrotum were present and an ecchymotic area was noted on the shaft of the penis. White blood count (WBC) was 17,900, with a differential count of 76 polymorphonuclear leukocytes, 13 bands and 11 lymphocytes. Hemoglobin was 12.5 gm. and hematocrit was 37.9 per cent. Platelets were 410,000. Urinalysis yielded 18 to 20 red blood cells, no casts and no protein. Throat culture was positive for group A beta-hemolytic streptococci. Antistreptolysin O titer was 1:250 Todd units. Diagnosis was torsion of the left testis and the patient was immediately taken to surgery. At operation edema and inflammation of the scrotum were noted and the appendix testis was acutely inflamed. No testicular torsion was found. The appendix testis was excised and microscopic examination revealed a diffuse polymorphonuclear infiltrate throughout its stroma. Convalescence was uneventful. Penicillin and 5 mg. prednisone 3 times daily were given. The patient was discharged from the hospital perfectly well 3 days later. Case 2. A 6-year-old white boy was admitted to Children's Memorial Hospital with a history of hives. The hives were red and reddish-brown, large, slightly raised and minimally pruritic lesions on the arms, chest, abdomen and ankles, which faded and reappeared several times during the month before hospitalization. He had no joint symptoms, abdominal pain or fever. Treatment of the rash with antihistamines failed to produce any improvement. Petechiae developed behind the knees 4 days before hospitalization. The next day, numerous petechiae were present on the dorsum of both feet and overlying the ankles, along with large ecchymotic areas. The patient complained that his ankles hurt but they were not swollen. On the day of hospitalization a large, purple ecchymosis appeared on the shaft of the penis and during the next few hours, the penis and scrotum rapidly became swollen and hemorrhagic. There was no history of trauma to the genitalia. Also, a new crop of petechiae appeared on the buttocks and perineum. The boy had the aforementioned petechial rash on the buttocks, perineum and lower extremities, and a huge, swollen, ecchymotic penis and scrotum (fig. 2). Temperature was 37.6C orally, pulse 100 and blood pressure 100/70. The left tympanic membrane was dull and thickened, and the pharnyx was injected. The scrotum was firm and non-tender, and the testes could not be palpated. No joint swelling or tenderness was noted. The WBC was 9,500, with a differenital count of 76 polymorphonuclear leukocytes, 22 lymphocytes and 2 mono-

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cytes. Hemoglobin was 14.6 gm. and hematocrit was 44.6 per cent. The platelet count was 370,000. Urinalysis, blood urea nitrogen, creatinine, creatinine clearance and beta-1-c globulin were normal. Throat culture for beta-hemolytic streptococci was negative. The stool was hematest negative for blood. An ice pack was placed on the scrotum, and the swelling and hemorrhage of the genitalia did not progress. The next day the scrotum was softer and the testicles could be palpated. There was no evidence of testicular swelling or tenderness. The patient was given penicillin and pseudoephedrine hydrochloride for otitis media, and was discharged from the hospital, improved, after 5 days of symptomatic therapy for the scrotal swelling. DISCUSSION

FIG. 1. Case 1. Purpuric lesions over buttocks

F1G. 2. Case 2. Hemorrhage of scrotum and penis

Our 2 patients presented with unusual manifestations of the Schonlein-Henoch syndrome. Although the disease may occur at any age from infancy through adulthood, the greatest incidence is in patients between 3 and 7 years old. Male subjects are affected almost twice as frequently as female subjects. A variety of allergenic agents ranging from foods"· 9 to bee stings 10 to specific bacteria,•· 1 1 • 12 particularly the group A beta-hemolytic streptococcus, 13 • 14 have been implicated etiologically. Attempts to confirm these relationships have yielded conflicting results. 1 • 15 - 17 Some investigators have conclude'd that an association of the streptococcus with this syndrome could not be invoked in any statistically significant proportion of cases. 18 Children have been reported to have abdominal pain and petechiae over the lower extremities immediately after exposure to cold temperatures or snow and after ingestion of ice cold drinks. 19• 20 The degree of scrotal hemorrhage that our second patient had is rare since it is an uncommon complication of this syndrome. Sahn and Schwartz reported an incidence of 38 per cent in 20 cases. 7 Few other reports of testicular involvement have been published in the literature. Fitzsimmons described 3 young boys who had testicular pain and swelling clinically, simulating acute testicular torsion. 4 Five similar cases were reported by Noussias and associates. 21 Some of these patients were explored surgically and were discovered to have vasculitis of the testis or appendix testis but none was complicated by testicular torsion. This led to the opinion that surgical exploration should be avoided in the child who presents, as did our first child, with symptoms of testicular torsion during the course of anaphylactoid purpura. This view has had to be revised in the light of more recent studies. Loh and Jalan described a boy who presented with the Schonlein-Henoch syndrome and who, in a matter of hours, had acute pain and swelling in the scrotum. 22 Exploration of the scrotum revealed vasculitis of the testis characteristic of anaphylactoid purpura but an acute torsion of the testis was also found, which, when relieved, promptly regained a normal blood supply. Vasculitis with edema and hemorrhage in the testis has been suggested as a predisposing cause of torsion just as vasculitis predisposes to intussusception in the gastrointestinal tract. 13 The diagnosis of testicular torsion is difficult to make when it is complicated by vasculitis of the testis. Both conditions can occur simultaneously. Whenever acute testicular pain and swelling occur in the course of the Schonlein-Henoch syndrome, torsion of the testis must be considered and surgical exploration performed. However, if the testes can be palpated and are noted not to be tender or enlarged, a more conservative approach can be followed, knowing that most of these patients have involvement primarily of the scrotum. Dr. Allan D. Schwartz critically read this manuscript, and Drs. Thomas Neglia and Bruce Berget assisted in the care of these patients.

SCROTAL SWELLING IN SCHONLEIN-HENOCH SYNDROME REFERENCES

1. Allen, D. M., Diamond, L. K. and Howell, D. A.: Anaphylactoid purpura in children. (Schonlein-Henoch syndrome): a review with a follow-up of the renal complications. Amer. J. Dis. Child., 99: 833, 1960. 2. Wedgewood, R. J.P. and Klaus, M. H.: Anaphylactoid purpura (Schonlein-Henoch syndrome); a long-term follow-up study with special reference to renal involvement. Pediatrics, 16: 196, 1955. 3. Silber, D. L.: Henoch-Schoenlein syndrome. Pediat. Clin. N. Amer., 19: 1061, 1972. 4. Fitzsimmons, J. S.: Uncommon complication of anaphylactoid purpura. Brit. Med. J., 4: 431, 1968. 5. Turpin, J.-C., Malpuech, G., Lavignon, A. and Demontis, B.: Les manifestations neurologiques du purpura rhumatoide. Pediatrie, 28: 185, 1973. 6. Roy, S.: Steatorrhoea in Henoch's syndrome. Brit. Med. J., 3: 682, 1972. 7. Sahn, D. J. and Schwartz, A. D.: Schonlein-Henoch syndrome: observations on some atypical clinical presentations. Pediatrics, 49: 614, 1972. 8. Ackroyd, J. F.: Allergic purpura, including purpura due to foods, drugs and infections. Amer. J. Med., 14: 605, 1953. 9. Alexander, H. L. and Eyermann, C. H.: Food allergy in Henoch's purpura. Arch. Dermat. Syph., Hi: 322, 1927. 10. Sharan, G., Anand, R. K. and Sinha, K. P.: Schonlein-Henoch syndrome after insect bite. Brit. Med. J., 1: 656, 1966. 11. Bauch, S.: Three cases of purpura hemorrhagica in chronic tuberculosis. Arch. Intern. Med., 17: 444, 1916. 12. Coke, H.: Two interesting cases of purpura. Brit. Med. J., l: 535, 1931.

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13. Gairdner, D.: The Schi:inlein-Henoch syndrome (anaphylactoid purpura). Quart. J. Med., 17: 95, 1948. 14. Smith, R. and Bertram, T. A.: Purpura with intense abdominal pain as a late complication of scarlet fever. Canad. Med. Ass. J., 16: 555, 1926. 15. Bywaters, E. G., Isdale, I. and Kempton, J. J.: Schi:inlein-Henoch purpura: evidence of group A ,B-haemolytic streptococcal aetiology. Quart. J. Med., 26: 161, 1957. 16. Sterky, G. and Thilen, A.: A study on the onset and prognosis of acute vascular purpura (the Schonlein-Henoch syndrome) in children. Acta Paediat., 49: 217, 1960. 17. Vernier, R. L., Worthen, H. G., Peterson, R. D., Colle, E. and Good, R. A.: Anaphylactoid purpura. I. Pathology of the skin and kidney and frequency of streptococcal infection. Pediatrics, 27: 181, 1961. 18. Ayoub, E. M. and Hoyer, J.: Anaphylactoid purpura: streptococcal antibody tite?s and fJ le-globulin levels. J. Pediat., 75: 193, 1969. 19. Peters, G. A. and Horton, B. T.: Allergic purpura with special reference to hypersensitiveness to cold. Proc. Mayo Clin. Staff Meet., Hi: 631, 1941. 20. Rogers, P. W., Bunn, S. M., Jr., Kurtzman, N. A. and White, M. G.: Schonlein-Henoch syndrome associated with exposure to cold. Arch. Intern. Med., 128: 782, 1971. 21. Noussias, M., Blandy, A. C. and Ward-McQuaid, M.: Intussusception in Henoch-Schi:inlein purpura. A report of two cases requiring operation. Brit. J. Surg., 56: 503, 1969. 22. Loh, H. S. and Jalan, 0. M.: Testicular torsion in Henoch-Schonlein syndrome. Brit. Med. J., 2: 96, 1974.

Scrotal swelling in the Schönlein-Henoch syndrome.

Testicular and scrotal hemorrhage is uncommon in the Schönlein-Henoch syndrome. The diagnosis of testicular torsion is difficult to make when it is co...
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