- Email: [email protected]
Idiopathic colitis associated with scrotal cellulitis
Volume 94 Number 3
B r i e f clinical and laboratory observations
by The Rehabilitation Center of the New York University Medical Center. ~ They point out that the rate of loss of vital capacity slows once it has fallen below one liter. We saw dramatic restoration of activity in our patients after an initial period of aggressive treatment. Although b o u n d to wheelchairs, Patients 1 and 5 have been able to resume their daily activities. They were not ready to die, and they and their families are grateful that they were given the option not to do so. Our patient with centronuclear myopathy is able to walk between classes without a wheelchair. All three of these young adults use their respirators only at night. We urge that any physician caring for children with neuromuscular disease include pulmonary function testing as part of the periodic examination. The option of ventilatory support should be thoroughly understood by patient, parent, and physician. Patients may wish to consider elective fitting of a cuirass prior to the onset of respiratory failure. Frequently, nighttime use may be all that is necessary. A cuirass seems to fit the unique needs of the patient with neuromuscular diseaseS: mobility, preservation of voice, and independence.
42 1
2. Plum F, and Lukes DS: An evaluation of the cuirass respirator in acute poliomyelitis with respiratory insufficiency, Am J Med Sci 121:417, 1951. 3. Wiers PWJ, LeCoultre R, Dallinga OT, Van Dijl W, Meinesz AF, and Sluiter H J: Cuirass respirator treatment of chronic respiratory failure in scoliotic patients, Thorax 32:221, 1977. 4. Stern L, Ramos AD, Outerbridge EW, and Beaudry PH: Negative pressure artifical respiration: Use in treatment of respiratory failure of the newborn, Can Med Assoc J 102:595, 1970. 5. Riley D J, Santiago TV, Daniele RP, Schall B, and Edelman NH: Blunted respiratory drive in congenital myopathy, Am J Med 63:459, 1977. 6. Johnson EW, and Kennedy JH: Comprehensive management of Duchenne's muscular dystrophy, Arch Phys Med Rehabil 52:110, 1971. 7. Aberion G, Alba A, Lee MHM, and Solomon M: Pulmonary care of Duchenne type muscular dystrophy, NY State J Med 73:1206, 1973. 8. Council on Physical Medicine: Tentative requirements for acceptance of respirators of the cuirass type, JAMA 135:715, 1947. 9. Liebhaber M, Lewiston N, and Robin E: Reye's syndrome complicated by Ondine's curse, West J Med 126:110, 1977.
REFERENCES I. Council on Physical Medicine: Monaghan portable respirator acceptable, JAMA 139:1273, 1949.
Idiopathic colitis associated with scrotal cellulitis Brian D. O'Brien, M.D., Alan B.R. Thomson, M.D., Ph.D., F.R.C.P.(C),* D. Lorne Tyrrell, M.D., Ph.D., F.R.C.P.(C), and Lawrence D. Jewell, M.D., F.R.C.P.(C), Edmonton, Alberta, Canada
A VARZETY of dermatologic manifestations of inflammatory bowel disease have been reported, including erythema nodosum, pyoderma gangrenosum, and nonspecific eczematous skin rashes? We describe a case of Fournier scrotal cellulitis associated with an idiopathic colitis. CASE REPORT
A 14-year-old boy developed sudden diffuse scrotal swelling with marked tenderness. He had no urinary symptoms and denied trauma to the affected area. In the preceding 14 months he had had intermittent diarrhea lasting about six days each month. The stools were poorly formed and contained small amounts of bright red blood. Two years prior he had had an episode of non-B hepatitis, which resolved without complication; From the Departments of Medicine and Pathology, University o f A lberta. *Reprint address: 9-I 12 Clinical Sciences Building, University of Alberta, Edmonton, Alberta, T6G 2G3.
0022-3476/79/300421 +03500.30/0 9 1979 The C. V. Mosby Co.
his past history was otherwise unremarkable. No other family members had symptoms of bowel disorders. On physical examination he was afebrile and at the sixtieth percentile for height and weight. Abnormal findings on examination included a reduced chest expansion of 3 cm, reduced lumbar spine extension of 5 cm on full flexion, and a tender, ill-defined fullness in the right iliac fossa. There was diffuse tender swelling of the entire scrotal wall and reddish purple discoloration of the scrotal skin. Laboratory evaluation showed: erythrocyte sedimentation rate 24 mm/hour; hemoglobin 10 gm/dl; mean corpuscular volume 77 fl; serum iron 22/tg/dl; iron binding capacity 418/~g/dl; and serum folate 1.8 ng/ml. A peripheral smear revealed some microcytic hypochromic ceils, and a bone marrow smear showed absent stainable iron stores. Normal studies included urinalysis, serum vitamin B~2, and stool cultures. Aerobic and anaerobic cultures of blood and urine were sterile. Sigmoidoscopic examination demonstrated focal areas of friability, tiny mucosal ulcerations with surrounding erythema, and a
422
Brief clinical and laboratory observations
normal-appearing intervening mucosa. Biopsy from the abnormal portion of rectal mucosa showed histologic changes of severe mucosal inflammation, with diffuse mucus depletion of rectal glands and a marked increase in lamina propria lymphocytes and plasma cells, with few neutrophils. There were many prominent lymphoid aggregates in the mucosa with some extension into the superficial submucosa. There were no granulomata or crypt abscesses. Radiologic examination of the colon showed a loss of the normal haustral markings and superficial ulceration from the mid-descending colon to the rectum. X-ray studies of the kidneys, bladder, and urethra, and the upper gastrointestinal tract including the terminal ileum, were normal: The sacroiliac joints were normal on routine x-ray examination and on bone scan. On the basis of the low serum folate concentration and the iron deficiency anemia, a small bowel biopsy was performed. This showed nonspecific changes in the mucosal epithelial ceils and a slight increase in chronic inflammatory cells in the lamina propria. Assessment of cell-mediated immunity by standard techniques showed a reduction in T cell number, an increase in K cells, and a normal number of B cells. Lymphocyte transformation after stimulation with phytohemagglutinin, concanavalin A, and varidase showed normal responses; K-cell activity was also within the normal range. Forty-eight hours after starting intravenous treatment with penicillin, gentamicin, and chloramphenicol, there was marked improvement in the scrotal swelling and discoloration; by four days all scrotal abnormalities had vanished. Treatment was then begun with sulphasalazine 2 gm daily, and within l0 days the diarrhea had resolved; he received no prednisone. During the next two years he had no recurrence of scrotal problems. The inflammatory bowel disease was treated for one year with the same dose of sulphasalazine; he gained 5 kg, continued to remain free of diarrhea, and experienced a return of his normal sense of well-being. The anemia responded fully to parenteral treatment with iron. Twenty months after stopping sulphasalazine, he had no recurrence of any symptoms, and sigmoidoscopy, rectal mucosal biopsy, barium enema, and enteroclysis were entirely normal. DISCUSSION The clinical pattern in this boy was that of a superficial scrotal infection described as Fournier syndrome, with idiopathic colitis. Fournier syndrome is a gangrenous infection of the scrotal tissues, first described in 1884. 2" Although predisposing factors may include urethral and scrotal trauma, perianal infections and abscesses, and fistulas between areas of diverticulitis and the bladder, many cases appear to be spontaneous. The youngest previously reported individual with the syndrome was 22 years of age. The major causative organisms are enteric aerobes and anaerobes? In most patients the scrotal skin is involved, leaving the epididymis and the testes intact. The infection can spread rapidly to the thigh, perineum, and anterior abdominal wall. Treatment is most successful with aggressive high-dose parenteral antibiotic therapy directed against the usual organisms, with early surgical
The Journal of Pediatrics March 1979
debridement if required. Our patient's rapid response to antibiotics supported the infectious origin of the inflammation and made debridement unnecessary. No intradermal or subcutaneous cultures were obtained from the scrotum because of the fear that this would cause ulceration or spread of the infection. The cause of his inflammatory bowel disease is not known. Cultures were negative for ameba, shigella, and Yersinia enterocolitica. There was no evidence of ileal involvement characteristic of Yersinia infections. Attempts to culture Campylobacter were not carried ouk but this is an unlikely causative organism because the clinical features of fever and explosive bloody diarrhea were not present? The patchy nature of the mucosal abnormality, the absence of profuse rectal bleeding or acute inflammatory cells in the biopsy, and the complete clinical remission for a two-year period of follow-up make the diagnosis of chronic ulcerative colitis unlikely. Some features are similar to a new syndrome described by Bonfils as "acute spontaneously recovering colitis." However, our patient did not have the characteristic fever, had diarrhea over a longer period, and had rectal mucosai ulcerations. 7 Crohn disease was initially considered as a possible diagnosis. Evidence supporting this included the patchy nature of the mucosal ulceration seen on sigmoidoscopy, the degree of mucosal and submucosal inflammation histologically, the anemia and low serum-folate values, and the nonspecific changes in the jejunal mucosa seen in the small bowel biopsy. However, the normal small bowel radiographs, the absence of granulomas, and the complete remission for two years are against this diagnosis? Despite this, it is possible that our patient has a variant of Crohn colitis. The cessation of the diarrhea in our patient may have been the result of antibiotics eliminating an etiologic organism or changing the bowel flora. The occurrence of scrotal cellulitis and inflammatory bowel disease may be coincidental, but the history of diarrhea and the simultaneous disappearance of the scrotal and bowel lesions with antibiotic therapy strongly suggest an association. Thus, when scrotal cellulitis occurs spontaneously in children, underlying inflammatory bowel disease should be excluded as a predisposing factor. Thanks are due to Dr. J.O. Metcalfe for referral of the patient and to Dr. J. Lyanga and P. Davis for performing the tests of cell-mediated immunity, to Dr. J.B. Dossetor for determining the tissue typing, Drs. W.M. Weinstein and P. Dolan for critically reviewing the manuscript, and to Ms. K. Penner for assistance in preparing the manuscript. REFERENCES
1. Greenstein AJ, Janowitz HD, and Sachar DB" The extraintestinal complications of Crohn's disease and ulcerative colitis: A study of 700 patients, Medicine 55:401, 1976.
Volume 94 Number 3
Brief clinical and laboratory observations
2. Devroede G, Schlaedor G, Sanches G, et al: Crohn's disease of the vulva, Am J Clin Pathol 63:348, 1975. 3. Foumier FA: Etude cliniquc de la gangrene trouve autour de la verge, Sem Med 4:619, 1884. 4. Rudolph R, Soloway M, DePalma RG, et ah Fournier's syndrome: Synergistic gangrene of the scrotum, Am J Surg 129:591, 1975.
423
5. Burpee JF, and Edwards P: Fournier's gangrene, J Urol 107:812, 1972. 6. Lyanga JJ, Davis P, and Thomson ABR: Alterations in cell-mediated immunity in patients with Crohn's disease and ulcerative colitis, (submitted for publication). 7. Finegold SM: Anaerobic bacteria in human disease, New York, 1977, Academic Press Inc., pp 411-416.
Parainfluenza pneumonia in severe combined immunodeficiency disease William R. Jarvis, M.D., Peter J. Middleton, M.D., and Erwin W. Geifand, M.D.,* Toronto, Ont., Canada
IN PATIENTS with severe combined i m m u n e deficiency, death is often secondary to pneumonitis. Most reports refer to Pneumocystis carinii or bacteria as the c o m m o n pathogen, whereas viral infections are rarely described as playing a major role in these patients. This paper describes two infants with S C I D who developed parainfluenza virus pneumonitis, ending in respiratory failure and death. CASE REPORTS Patient 1. This 9-week-old white girl was transferred to the Hospital for Sick Children for evaluation following two episodes of possible neonatal sepsis. Fever, poor feeding, and lethargy were observed during the first days of life. All cultures for bacteria were negative and, after completing a course of antibiotics, she was discharged. At five weeks of age she was admitted for treatment of possible osteomyelitis. Laboratory investigations revealed abnormally low serum immunoglobulin levels and impaired T-cell function, and she was transferred to HSC for evaluation. On admission she was found to be less than the third percentile for both length and weight. Physical examination revealed an absence of lymphoid tissue, hypertonia, and multiple inflamed iv sites on the head and extremities. Immunologic evaluation confirmed a combined humoral and cellular immune deficiency with profound hypogammaglobulinemia, less than 10% E-rosetring T-lymphocytes, and absent proliferative responses to phytohemagglutinin, concanavalin A, pokeweed mit0gen, and allogeneic cells. Parenteral gamma globulin replacement was begun. On admission a chest radiograph revealed bilateral upper lobe consolidation. The initial nasopharyngeal aspirate revealed
From the Departments of Virology and Immunology, The Hospital for Sick Children. *Reprint address: Departmentof lmmunolog),, The Hospital for Sick Children, 555 UniversityAve., Toronto, Ont., M5G 1)(8. 0022-3476/79/300423 + 03500.30/0 9 1979 The C. V. Mosby Co.
parainfluenza type 3 in cell culture. Ten days after admission she developed tachypnea and pyrexia; over the following 289 weeks, respiratory symptoms progressed. Hypoxia supervened, requiring supplemental oxygen. A tracheal aspirate obtained by direct laryngoscopy was negative for bacteria, fungi, and Pneumocystis carinii. Para 3 continued to be present in this as well as other respiratory secretions. Abbreviations used SCID: severe combined immune deficiency HSC: Hospital for Sick Children Para 3: parainfluenza type 3 Chest radiographs then revealed increasing consolidation and a large pleural effusion; thoracentesis was performed and was negative in culture for bacteria, fungi, and viruses. Because of continued deterioration and increasing oxygen requirement, an open lung biopsy was performed. Electron microscopy showed a paramyxovirus (Fig. 1) later identified as Para 3 by cell culture isolation. Lung biopsy cultures for bacteria, fungi, as well as stains for Pneumocystis and acid-fast bacteria were negative. Intubation was required but hypoxia and hypercapnea persisted; chest radiographs revealed complete consolidation of the right lung and increasing consolidation of the left lung. A cardiac arrest ensued and resuscitation attempts failed. Viral cultures of nasopharyngeal secretions from the last five days of life all revealed Para 3 in cell culture. Autopsy findings were consistent with the diagnosis of SCID, with marked lymphoid hypoplasia, thymic dysplasia, and the absence of Hassall corpuscles. The lungs showed focal pulmonary hemorrhage, alveolar cell hyperplasia and hypertrophy, and distended alveoli filled with eosinophilic proteinaceous material (Fig. 2). All postmortem bacterial cultures were negative. Para 3 was isolated from the trachea, nasopharynx, and lung. Patient 2. This 2-month-old white girl was transferred to HSC for evaluation of recurrent infections. From day 12 of age the patient had had upper respiratory tract symptoms and localized skin infections. A chest radiograph revealed pneumonia, for which she was treated with antibiotics and discharged. Symptoms
B r i e f clinical and laboratory observations
by The Rehabilitation Center of the New York University Medical Center. ~ They point out that the rate of loss of vital capacity slows once it has fallen below one liter. We saw dramatic restoration of activity in our patients after an initial period of aggressive treatment. Although b o u n d to wheelchairs, Patients 1 and 5 have been able to resume their daily activities. They were not ready to die, and they and their families are grateful that they were given the option not to do so. Our patient with centronuclear myopathy is able to walk between classes without a wheelchair. All three of these young adults use their respirators only at night. We urge that any physician caring for children with neuromuscular disease include pulmonary function testing as part of the periodic examination. The option of ventilatory support should be thoroughly understood by patient, parent, and physician. Patients may wish to consider elective fitting of a cuirass prior to the onset of respiratory failure. Frequently, nighttime use may be all that is necessary. A cuirass seems to fit the unique needs of the patient with neuromuscular diseaseS: mobility, preservation of voice, and independence.
42 1
2. Plum F, and Lukes DS: An evaluation of the cuirass respirator in acute poliomyelitis with respiratory insufficiency, Am J Med Sci 121:417, 1951. 3. Wiers PWJ, LeCoultre R, Dallinga OT, Van Dijl W, Meinesz AF, and Sluiter H J: Cuirass respirator treatment of chronic respiratory failure in scoliotic patients, Thorax 32:221, 1977. 4. Stern L, Ramos AD, Outerbridge EW, and Beaudry PH: Negative pressure artifical respiration: Use in treatment of respiratory failure of the newborn, Can Med Assoc J 102:595, 1970. 5. Riley D J, Santiago TV, Daniele RP, Schall B, and Edelman NH: Blunted respiratory drive in congenital myopathy, Am J Med 63:459, 1977. 6. Johnson EW, and Kennedy JH: Comprehensive management of Duchenne's muscular dystrophy, Arch Phys Med Rehabil 52:110, 1971. 7. Aberion G, Alba A, Lee MHM, and Solomon M: Pulmonary care of Duchenne type muscular dystrophy, NY State J Med 73:1206, 1973. 8. Council on Physical Medicine: Tentative requirements for acceptance of respirators of the cuirass type, JAMA 135:715, 1947. 9. Liebhaber M, Lewiston N, and Robin E: Reye's syndrome complicated by Ondine's curse, West J Med 126:110, 1977.
REFERENCES I. Council on Physical Medicine: Monaghan portable respirator acceptable, JAMA 139:1273, 1949.
Idiopathic colitis associated with scrotal cellulitis Brian D. O'Brien, M.D., Alan B.R. Thomson, M.D., Ph.D., F.R.C.P.(C),* D. Lorne Tyrrell, M.D., Ph.D., F.R.C.P.(C), and Lawrence D. Jewell, M.D., F.R.C.P.(C), Edmonton, Alberta, Canada
A VARZETY of dermatologic manifestations of inflammatory bowel disease have been reported, including erythema nodosum, pyoderma gangrenosum, and nonspecific eczematous skin rashes? We describe a case of Fournier scrotal cellulitis associated with an idiopathic colitis. CASE REPORT
A 14-year-old boy developed sudden diffuse scrotal swelling with marked tenderness. He had no urinary symptoms and denied trauma to the affected area. In the preceding 14 months he had had intermittent diarrhea lasting about six days each month. The stools were poorly formed and contained small amounts of bright red blood. Two years prior he had had an episode of non-B hepatitis, which resolved without complication; From the Departments of Medicine and Pathology, University o f A lberta. *Reprint address: 9-I 12 Clinical Sciences Building, University of Alberta, Edmonton, Alberta, T6G 2G3.
0022-3476/79/300421 +03500.30/0 9 1979 The C. V. Mosby Co.
his past history was otherwise unremarkable. No other family members had symptoms of bowel disorders. On physical examination he was afebrile and at the sixtieth percentile for height and weight. Abnormal findings on examination included a reduced chest expansion of 3 cm, reduced lumbar spine extension of 5 cm on full flexion, and a tender, ill-defined fullness in the right iliac fossa. There was diffuse tender swelling of the entire scrotal wall and reddish purple discoloration of the scrotal skin. Laboratory evaluation showed: erythrocyte sedimentation rate 24 mm/hour; hemoglobin 10 gm/dl; mean corpuscular volume 77 fl; serum iron 22/tg/dl; iron binding capacity 418/~g/dl; and serum folate 1.8 ng/ml. A peripheral smear revealed some microcytic hypochromic ceils, and a bone marrow smear showed absent stainable iron stores. Normal studies included urinalysis, serum vitamin B~2, and stool cultures. Aerobic and anaerobic cultures of blood and urine were sterile. Sigmoidoscopic examination demonstrated focal areas of friability, tiny mucosal ulcerations with surrounding erythema, and a
422
Brief clinical and laboratory observations
normal-appearing intervening mucosa. Biopsy from the abnormal portion of rectal mucosa showed histologic changes of severe mucosal inflammation, with diffuse mucus depletion of rectal glands and a marked increase in lamina propria lymphocytes and plasma cells, with few neutrophils. There were many prominent lymphoid aggregates in the mucosa with some extension into the superficial submucosa. There were no granulomata or crypt abscesses. Radiologic examination of the colon showed a loss of the normal haustral markings and superficial ulceration from the mid-descending colon to the rectum. X-ray studies of the kidneys, bladder, and urethra, and the upper gastrointestinal tract including the terminal ileum, were normal: The sacroiliac joints were normal on routine x-ray examination and on bone scan. On the basis of the low serum folate concentration and the iron deficiency anemia, a small bowel biopsy was performed. This showed nonspecific changes in the mucosal epithelial ceils and a slight increase in chronic inflammatory cells in the lamina propria. Assessment of cell-mediated immunity by standard techniques showed a reduction in T cell number, an increase in K cells, and a normal number of B cells. Lymphocyte transformation after stimulation with phytohemagglutinin, concanavalin A, and varidase showed normal responses; K-cell activity was also within the normal range. Forty-eight hours after starting intravenous treatment with penicillin, gentamicin, and chloramphenicol, there was marked improvement in the scrotal swelling and discoloration; by four days all scrotal abnormalities had vanished. Treatment was then begun with sulphasalazine 2 gm daily, and within l0 days the diarrhea had resolved; he received no prednisone. During the next two years he had no recurrence of scrotal problems. The inflammatory bowel disease was treated for one year with the same dose of sulphasalazine; he gained 5 kg, continued to remain free of diarrhea, and experienced a return of his normal sense of well-being. The anemia responded fully to parenteral treatment with iron. Twenty months after stopping sulphasalazine, he had no recurrence of any symptoms, and sigmoidoscopy, rectal mucosal biopsy, barium enema, and enteroclysis were entirely normal. DISCUSSION The clinical pattern in this boy was that of a superficial scrotal infection described as Fournier syndrome, with idiopathic colitis. Fournier syndrome is a gangrenous infection of the scrotal tissues, first described in 1884. 2" Although predisposing factors may include urethral and scrotal trauma, perianal infections and abscesses, and fistulas between areas of diverticulitis and the bladder, many cases appear to be spontaneous. The youngest previously reported individual with the syndrome was 22 years of age. The major causative organisms are enteric aerobes and anaerobes? In most patients the scrotal skin is involved, leaving the epididymis and the testes intact. The infection can spread rapidly to the thigh, perineum, and anterior abdominal wall. Treatment is most successful with aggressive high-dose parenteral antibiotic therapy directed against the usual organisms, with early surgical
The Journal of Pediatrics March 1979
debridement if required. Our patient's rapid response to antibiotics supported the infectious origin of the inflammation and made debridement unnecessary. No intradermal or subcutaneous cultures were obtained from the scrotum because of the fear that this would cause ulceration or spread of the infection. The cause of his inflammatory bowel disease is not known. Cultures were negative for ameba, shigella, and Yersinia enterocolitica. There was no evidence of ileal involvement characteristic of Yersinia infections. Attempts to culture Campylobacter were not carried ouk but this is an unlikely causative organism because the clinical features of fever and explosive bloody diarrhea were not present? The patchy nature of the mucosal abnormality, the absence of profuse rectal bleeding or acute inflammatory cells in the biopsy, and the complete clinical remission for a two-year period of follow-up make the diagnosis of chronic ulcerative colitis unlikely. Some features are similar to a new syndrome described by Bonfils as "acute spontaneously recovering colitis." However, our patient did not have the characteristic fever, had diarrhea over a longer period, and had rectal mucosai ulcerations. 7 Crohn disease was initially considered as a possible diagnosis. Evidence supporting this included the patchy nature of the mucosal ulceration seen on sigmoidoscopy, the degree of mucosal and submucosal inflammation histologically, the anemia and low serum-folate values, and the nonspecific changes in the jejunal mucosa seen in the small bowel biopsy. However, the normal small bowel radiographs, the absence of granulomas, and the complete remission for two years are against this diagnosis? Despite this, it is possible that our patient has a variant of Crohn colitis. The cessation of the diarrhea in our patient may have been the result of antibiotics eliminating an etiologic organism or changing the bowel flora. The occurrence of scrotal cellulitis and inflammatory bowel disease may be coincidental, but the history of diarrhea and the simultaneous disappearance of the scrotal and bowel lesions with antibiotic therapy strongly suggest an association. Thus, when scrotal cellulitis occurs spontaneously in children, underlying inflammatory bowel disease should be excluded as a predisposing factor. Thanks are due to Dr. J.O. Metcalfe for referral of the patient and to Dr. J. Lyanga and P. Davis for performing the tests of cell-mediated immunity, to Dr. J.B. Dossetor for determining the tissue typing, Drs. W.M. Weinstein and P. Dolan for critically reviewing the manuscript, and to Ms. K. Penner for assistance in preparing the manuscript. REFERENCES
1. Greenstein AJ, Janowitz HD, and Sachar DB" The extraintestinal complications of Crohn's disease and ulcerative colitis: A study of 700 patients, Medicine 55:401, 1976.
Volume 94 Number 3
Brief clinical and laboratory observations
2. Devroede G, Schlaedor G, Sanches G, et al: Crohn's disease of the vulva, Am J Clin Pathol 63:348, 1975. 3. Foumier FA: Etude cliniquc de la gangrene trouve autour de la verge, Sem Med 4:619, 1884. 4. Rudolph R, Soloway M, DePalma RG, et ah Fournier's syndrome: Synergistic gangrene of the scrotum, Am J Surg 129:591, 1975.
423
5. Burpee JF, and Edwards P: Fournier's gangrene, J Urol 107:812, 1972. 6. Lyanga JJ, Davis P, and Thomson ABR: Alterations in cell-mediated immunity in patients with Crohn's disease and ulcerative colitis, (submitted for publication). 7. Finegold SM: Anaerobic bacteria in human disease, New York, 1977, Academic Press Inc., pp 411-416.
Parainfluenza pneumonia in severe combined immunodeficiency disease William R. Jarvis, M.D., Peter J. Middleton, M.D., and Erwin W. Geifand, M.D.,* Toronto, Ont., Canada
IN PATIENTS with severe combined i m m u n e deficiency, death is often secondary to pneumonitis. Most reports refer to Pneumocystis carinii or bacteria as the c o m m o n pathogen, whereas viral infections are rarely described as playing a major role in these patients. This paper describes two infants with S C I D who developed parainfluenza virus pneumonitis, ending in respiratory failure and death. CASE REPORTS Patient 1. This 9-week-old white girl was transferred to the Hospital for Sick Children for evaluation following two episodes of possible neonatal sepsis. Fever, poor feeding, and lethargy were observed during the first days of life. All cultures for bacteria were negative and, after completing a course of antibiotics, she was discharged. At five weeks of age she was admitted for treatment of possible osteomyelitis. Laboratory investigations revealed abnormally low serum immunoglobulin levels and impaired T-cell function, and she was transferred to HSC for evaluation. On admission she was found to be less than the third percentile for both length and weight. Physical examination revealed an absence of lymphoid tissue, hypertonia, and multiple inflamed iv sites on the head and extremities. Immunologic evaluation confirmed a combined humoral and cellular immune deficiency with profound hypogammaglobulinemia, less than 10% E-rosetring T-lymphocytes, and absent proliferative responses to phytohemagglutinin, concanavalin A, pokeweed mit0gen, and allogeneic cells. Parenteral gamma globulin replacement was begun. On admission a chest radiograph revealed bilateral upper lobe consolidation. The initial nasopharyngeal aspirate revealed
From the Departments of Virology and Immunology, The Hospital for Sick Children. *Reprint address: Departmentof lmmunolog),, The Hospital for Sick Children, 555 UniversityAve., Toronto, Ont., M5G 1)(8. 0022-3476/79/300423 + 03500.30/0 9 1979 The C. V. Mosby Co.
parainfluenza type 3 in cell culture. Ten days after admission she developed tachypnea and pyrexia; over the following 289 weeks, respiratory symptoms progressed. Hypoxia supervened, requiring supplemental oxygen. A tracheal aspirate obtained by direct laryngoscopy was negative for bacteria, fungi, and Pneumocystis carinii. Para 3 continued to be present in this as well as other respiratory secretions. Abbreviations used SCID: severe combined immune deficiency HSC: Hospital for Sick Children Para 3: parainfluenza type 3 Chest radiographs then revealed increasing consolidation and a large pleural effusion; thoracentesis was performed and was negative in culture for bacteria, fungi, and viruses. Because of continued deterioration and increasing oxygen requirement, an open lung biopsy was performed. Electron microscopy showed a paramyxovirus (Fig. 1) later identified as Para 3 by cell culture isolation. Lung biopsy cultures for bacteria, fungi, as well as stains for Pneumocystis and acid-fast bacteria were negative. Intubation was required but hypoxia and hypercapnea persisted; chest radiographs revealed complete consolidation of the right lung and increasing consolidation of the left lung. A cardiac arrest ensued and resuscitation attempts failed. Viral cultures of nasopharyngeal secretions from the last five days of life all revealed Para 3 in cell culture. Autopsy findings were consistent with the diagnosis of SCID, with marked lymphoid hypoplasia, thymic dysplasia, and the absence of Hassall corpuscles. The lungs showed focal pulmonary hemorrhage, alveolar cell hyperplasia and hypertrophy, and distended alveoli filled with eosinophilic proteinaceous material (Fig. 2). All postmortem bacterial cultures were negative. Para 3 was isolated from the trachea, nasopharynx, and lung. Patient 2. This 2-month-old white girl was transferred to HSC for evaluation of recurrent infections. From day 12 of age the patient had had upper respiratory tract symptoms and localized skin infections. A chest radiograph revealed pneumonia, for which she was treated with antibiotics and discharged. Symptoms