CASE REPORT
Ulcerative Colitis and Anaphylactoid Purpura Robert M. Craig, MD, Michael Ashman, MD, Sheldon Feldman, MD, Junji Hasegawa, MD, and Erl Dordal, MD
Anaphylactoid purpura
( A P ) is t h o u g h t to be
a ] h y p e r s e n s i t i v i t y d i s e a s e , o c c u r r i n g m o s t l y in c h i l d r e n a n d y o u n g a d u l t s a n d c h a r a c t e r i z e d by e r y t h e m a t o - u r t i c a r i a l p a p u l e s a n d p u r p u r a in a p a t i e n t w i t h n o r m a l p l a t e l e t s . In a d d i t i o n to cutaneous involvement, the gastrointestinal tract, t h e k i d n e y s , t h e j o i n t s , a n d t h e l u n g s a r e also f r e q u e n t l y
involved,
particularly
in t h e
a d u l t (I). W h i l e t h e c o l o r e c t a l i n v o l v e m e n t is c h a r a c t e r i s t i c a l l y s e e n as b o w e l e c c h y m o s e s , t h e case p r e s e n t e d h e r e is b e l i e v e d to be t h e first w e l l - d o c u m e n t e d case o f u l c e r a t i v e colitis ( U C ) becoming manifest during exacerbations of AP.
CASE REPORT This 21-year-old male student was well until one and one half years prior to admission to the Veterans Administration Research Hospital when he had an episode of bloody diarrhea and abdominal cramping. Six months later he developed two flat erythematous patches on his legs termed "erythema marginatum." Raised purpurie'lesmns appeared on his buttocks, associated with swelling of his left wrist, 8 months prior to admission and 2 weeks after entering the U.S. Army. Subsequently, he had recurrent erythematous eruptions most often on his lower extremities and buttocks, associated with swelling of his hands, feet, ankles, and face. Occasionally, diarrhea and abdominal cramping would accompany the dermatologie and articular symptoms. He was informed that he had ulcerative colitis after one proctoscopic examination while in the mibtary. He was discharged from the Military Service 4 months prior to admission, but the recurrent skin lesions and polyarticular swelling occurred more frequently. One month prior to admission, his private physician placed him on 16 mg methylprednisolone, daily, which partially controlled his dermatologic and articular From the Gastroenterology and Dermatology Sections of the Northwestern University Medical School and the Veterans Administration Research Hospital, Chicago, Illinois. Address for reprint requests: Dr. Robert M. Craig, Gastroenterology Section, VA Hospital, 333 East Huron Street, Chicago, Illinois 60611.
Digestive Diseases, Yo~. 20, No. 5 (May ~g75)
symptoms, and he was gradually tapered to 4 mg daily. He admitted to have had frequent strep throats throughout his life, which had been treated with penicillin. There was no family history of angioneurotic edema, inflammatory bowel disease, or purpura. On his admission physical examination he was in no distress; b~ood pressure was ~34/80 mm Hg, pulse was 88/min and regular, and the temperature was 97.6" F. A l-cm erythematous nodule was present on his right lower leg. There were many hyperpigmented maeules on both legs. There was no lymphadenopathy and his spleen was not palpable. The remainder of his physical examination was normal. The urinalysis, complete blood count, and platelet count were normal on admission, but the hemoglobin fell to 11.0 g/100 ml during the hospitalization. The latex fixation was reactive initially but was negative after 1 week. LE preparations were negative. The ASP titer was 50 Todd units and serum complement was 73 hemolytic units per milliner (normal 40-50). The stools were positive for occult blood on muhiple occasions. Serum chemistries were normal with the exception of an alkaline phosphatase of 125 [U/liter (normal less than 85). The serum protein eleetrophoresis was normal. X-rays of his chest, upper-gastrointestinal tract, lower-gastrointestinal tract, wrists, hands, ankles, and knees were normal. A proctoseopic examination revealed multiple circumscribed erythematous lesions seen throughout the rectal mucosa. There was no friability. A biopsy of the rectum revealed small loci of mucosal hemorrhages and some acute inflammatory cells in the tuniea propria mucosae. A skin biopsy revealed leukoclastic angiitis. During this hospitalization he developed edema of his chest, scrotum, and face with elevated annular lesions on his trunk and extremities. Prednisone 30 mg daily was administered and gradually tapered, and his skin and gastrointestinal symptoms rapidly resolved. A follow-up proetoscopie examination was normal. During the 2 years after this initial hospitalization he had exacerbations of his skin lesions, sometimes associated with watery or bloody diarrhea, so that the dose of prednisone had to be adjusted. During one episode, he required rehospitalization for malaise, cough, occasional hemoptysis, and an associated 1S-pound weight loss, but no gastrointestinal symptoms. The physical examination revealed numerous 1- to 2-mm nonblanching erythematous papules and macules on his legs, buttocks, soles, and palms. His lungs were normal to percussion and auseuhation. The
487
CRAIG ET AL
,Fig 1. Representative area of colonic mucosa demonstrating cryptitis and crypt abscess formation consistent with ulcerative colitis (XlO0).
white blood count was 13,300/mm ~, with a normal differential. The eosinophile count r~anged between 0% and 5%. The hematocrit value was 35%. The platelet count was 365,000. Urinalysis and serum chemistries were normal, except for a persistently elevated alkaline phosphatase, ranging from 180 to 475 IU/liter and a leucine aminopeptidase of 313 Goldbarg-Rutenburg units per 0.02 ml. (normal 75-230). The chest x-ray film revealed bilateral diffuse infiltrates in both lung bases. Immunoelectrophoresis showed IgG, 2000 rag/100 ml (normal 1020 1460); IgA, 330 mg/ 100 ml (normal 210-350); and IgM 83 mg/100 ml (normal 85-155). Countercurrent electrophoresis for hepatitis B antigen was negative. Serum iron was 50 #g/100 ml and the iron binding capacity was 400 ~tg/100 ml. Intermediate strength PPD was negative. The patient was continued on prednisone and was treated for possible tuberculosis with isoniazid, ethambutal, and streptomycin. Subsequently, all sputum cultures were negative for mycobacteria. An oral cholecystogram was normal. A lung biospy was consistent with Loeffler's pneumonitis (The authors are grateful for the consultative opinion of Dr. Averell Liebow who con-
488
firmed the lung biopsy diagnosis). No immune deposits were seen by immunofluorescent techniques. A needle hiospy of the liver was normal. The patient's skin and pulmonary symptoms resolved rapidly, chest x-ray films became normal, and when discharged, isoniazid and prednisone therapy were continued. Over the subsequent year, he had only an occasional erythematous macule. The dose of prednisone was tapered and the patient did not develop diarrhea. Recurrent bloody diarrhea, numerous purpuric lesions on his buttocks, a lowgrade fever, and crampy abdominal pain occurred 15 months later, necessitating readmission to the hospital. On physical examination, temperature was 101 ~ F orally; there Was diffuse abdominal tenderness, with no signs of peritonitis; and scattered purpurie nonblanehing macules over the buttocks and both ankles. The hematoerit was 44 vol%; the WBC was 21,900/mm 3, with increased numbers of band forms. Urinalysis revealed microscopic hematuria but no albuminuria or casts. Creatinine clearance was 120 ml/ rain. The alkaline phosphatase was 300 IU/liter. Stools tested positive for occult blood, but no pathogens were seen
Digestive Diseases, Vol. 20, No. 5 (May 1975)
ULCERATIVE COLITIS AND ANAPHYLACTOID PURPURA
Fig 2. Higher-power magnification of Figure 1 (X 520).
or cultured. The chest x-ray study was normal. A proctoseopic examination revealed a markedly friable, diffuse rectosigmoid mucosa, actively bleeding, and extending beyond 25 cm. A rectal biopsy (Figures 1 and 2) demonstrated an active inflammatory mucosal process with crypt abscesses, consistent with ulcerative colitis. X-ray study of the colon showed loss of haustra in the transverse and descending coIons, and small ulcerations in the descending colon (Figure 3). The patient defervesced, and his skin and gastrointestinal symptoms rapidly resolved with prednisone therapy. He is presently maintained on prednisone as an outpatient. A foBow-up proctoscopic examination appeared normal, and a subsequent rectal mucosal biopsy was interpreted as normal. Digestive Diseases, Vol. 20, No. 5 (May 1975)
DISCUSSION T h e skin h i s t o p a t h o l o g y of a n a p h y l a c t o i d p u r p u r a ( A P ) is a n a r t e r i o l i t i s a n d c a p i l l a r i t i s w i t h e a r l y leukocytoclasis ( n u c l e a r dust) a n d e r y t h r o c y t e e x t r a v a s a t i o n (1). W h e n less active, a less florid, m i x e d c e l l u l a r or m o n o n u c l e a r vasculitis is p r e s e n t . T h e course is self limited in most cases. H o w e v e r , it m a y be r e c u r r e n t or c o n t i n u o u s in a few subjects, p a r t i c u l a r l y in t h e a d u l t (1). T h e histologic a n d clinical s y n d r o m e in t h e a d u l t is s i m i l a r to a n d m e r g e s w i t h hy489
CRAIG ET AL
Fig 3. X-ray of the colon demonstrating loss of haustra and small mucosal irregularities consistent with ulcerative colitis.
490
Digestive Diseases, Vol. 20, No. 5 (May 1975)
ULCERATIVE COLITIS AND ANAPHYLACTOID PURPURA
Fig 4. Representative skin lesions during exacerbation of anaphylactoid purpura.
persensitivity angiitis (2). The present case has typical histopathologic and clinical findings of AP of the recurrent variety. Figure 4 is a photograph of representative lesions during an exacerbation, typical of AP. Of interest was the associated ulcerative colitis found in our patient. Gastrointestinal manifestations of anaphylactoid purpura (AP) have been described frequently in the literature. Early descriptions of AP noted the frequent occurrence of abdominal colic, diarrhea and hematochezia in children (3,4). The relaDigestive Diseases, Vol. 20, No. 5 (May 1975)
tively high incidence of small-intestinal intussusception in this disorder has been described (5). Involvement of the small bowel has been elucidated further in reports on small bowel x-ray films showing localized dilation, or "thumb printing" (6-8). Colonic involvement in AP has been much less frequently described. Although hematochezia and diarrhea are not uncommonly noted in AP, particularly in adults (9), proctoscopic descriptions have been rarely presented, but when described, they were normal or demonstrated only multiple pc491
CRAIG ET AL
techiae (5, 10). There is one report of a patient with AP and a friable mucosa on proctoscopic examination, but no histologic corroboration of ulcerative colitis (UC) was presented (10). This is the first well-documented case the authors have been able to find of ulcerative colitis in an adult with typical AP. T h e diagnosis of ulcerative colitis in this case is substantiated by the diffuse proctosigmoiditis indistinguishable proctoscopically from ulcerative colitis, and the rectal biopsy identical to ulcerative colitis on histologic grounds. There was no evidence for amebic, bacillary, or tuberculous colitis. Further, independent observers, 2 years apart, considered the proctoseopic appearance typical of ulcerative colitis. Finally, the barium enema demonstrated findings consistent with ulcerative colitis. Although not proven by this case report, the occurrence of the two diseases in this patient does not appear to be a fortuitous association, because the skin and gastrointestinal manifestations presented simultaneously during exacerbations of the patient's illness. In addition, both the dermatologic and gastrointestinal aspects of his illness resolved in parallel with prednisone therapy. Although dermatologic disorders are frequently observed in ulcerative colitis, p u r p u r a have been described only rarely (11-14), and no clinical syndrome suggestive of AP has been described in association with ulcerative colitis. In addition to the skin and gastrointestinal aspects of this man's disorders, there were also renal and pulmonary complications. Although focal glomerulonephritis and rapidly progressive glomerulonephritis have been described with AP (10), the usual renal findings are microscopic hematuria, absent cylindruria, and normal renal function, as in this case (9). Biopsy of the' pulmonary infiltrates, demonstrating eosinophilia and vasculitis, is consistent with Loeffler's pneumonitis, and may be a reflection of a hypersensitivity phenomenon underlying the AP syndrome. T h e elevated alkaline phosphatase and leucine aminopeptidase in this patient cannot be readily explained. The 492
oral cholecystogram was normal, arguing against a diagnosis of bilary calculus disease. Unlike the liver biopsies of most patients with abnormal liver function tests and ulcerative colitis, the liver biopsy in the present case was normal, with no apparent pericholangitis (15), although only one portal field was present in the biopsy. Although this is the first well-documented case of ulcerative colitis reported in AP, the association may be a more common occurrence. Careful proctoscopic examinations with rectal biopsies should be performed and reported in patients with AP, especially in those with diarrhea or hematochezia.
SUMMARY
T h e first case of well-documented ulcerative colitis o c c u r r i n g d u r i n g e x a c e r b a t i o n s of anaphylactoid purpura has been presented. T h e patient experienced renal and pulmonary complications. Gastrointestinal manifestations of anaphylactoid purpura were reviewed. It has been advised that other patients with anaphylactoid purpura be carefully observed for evidence of ulcerative colitis.
REFERENCES
1. Wilkinson DS: Henoch-Schoenlein Syndrome, Textbook of Dermatology. Edited by A Rook, DS Wilkinson, and FSG Ebling. FA Davis Co, Philadelphia, 1968, pp 428429 2. Masters R, Herskovic T: Cutaneous manifestations of disorders of the alimentary tract. Dermatology in General Medicine. TB Fitzpatrick, FA Arndt, WH Clark, AZ Eisen, EJ Van Scott, J H Vaughn (eds). New York, McGraw-Hill, 1971, pp 1372-1373 3. Henoch I: Ueber eine eigenthuemliche form von purpura. Berl Klin Wehrschr 11:641-643, 1874 4. Osler W: On the visceral manifestations of the erythema group of skin diseases. Am J Med Sci 127:1-23, 1904 5. Feldt RH, Stikler GB: The gastrointestinal manifestations of anaphylactoid purpura in chilDigestive Diseases, Vol. 20, No. 5 (May 1975)
ULCERATIVE COLITIS AND ANAPHYLACTOID PURPURA
6.
7r.
8,.
9.
10.
dren. Proc Staff Meeting Mayo Clinic 37:465473, 1962 Whitemore WH, Paterson GH: Henoch's purpura: Small intestinal changes. Radiology 46:373-376, 1946 Fetter FS, Mills WL: Roentgenographic findings in Schoenlein-Henoch purpura. A case report. Radiology 55:545-547, 1950 Rodriguez-Erdmann F, Levitan R: Gastrointestinal and roentgenological manifestations of Henoch-Schoenlein purpura. Gastroenterology 260:260-264, 1960 Cream J J, Gumpel J M , Prachey R O G : Schoenlein-Henoch purpura in the adult. Q J Med 39:461484, 1970 Kraemer M: Henoch's purpura. A case with bullous skin lesions and residual scars, roent-
Digestive Diseases, Vol. 20, No. 5 (May 1975)
11.
12. 13.
14. 15.
genological considerations. Gastroenterology 9:608-610, 1947 Samitz MH, Greenberg MS: Skin lesions in association with ulcerative colitis. Gastroenterology 19:476479, 1951 Rice-Oxley JM, Truelove S: Complications of ulcerative colitis. Lancet 2:607-611, 1950 Hightower HC, Broders AC, Haines RD, McKenny JF, Sommer AW: Chronic ulcerative colitis. Am J Dig Dis 3:861-876, 1958 Johnson ML, Wilson HTH: Skirt lesions in ulcerative colitis. Gut 10:225-263, 1960 Dordal E, Glagov S, Kirsner JB: Hepatic lesions in chronic inflammatory bowel disease. I. Clinical correlations with liver biopsy diagnoses in 103 patients. Gastroenterology 52:239-253, 1967
493
Ulcerative Colitis and Anaphylactoid Purpura Robert M. Craig, MD, Michael Ashman, MD, Sheldon Feldman, MD, Junji Hasegawa, MD, and Erl Dordal, MD
Anaphylactoid purpura
( A P ) is t h o u g h t to be
a ] h y p e r s e n s i t i v i t y d i s e a s e , o c c u r r i n g m o s t l y in c h i l d r e n a n d y o u n g a d u l t s a n d c h a r a c t e r i z e d by e r y t h e m a t o - u r t i c a r i a l p a p u l e s a n d p u r p u r a in a p a t i e n t w i t h n o r m a l p l a t e l e t s . In a d d i t i o n to cutaneous involvement, the gastrointestinal tract, t h e k i d n e y s , t h e j o i n t s , a n d t h e l u n g s a r e also f r e q u e n t l y
involved,
particularly
in t h e
a d u l t (I). W h i l e t h e c o l o r e c t a l i n v o l v e m e n t is c h a r a c t e r i s t i c a l l y s e e n as b o w e l e c c h y m o s e s , t h e case p r e s e n t e d h e r e is b e l i e v e d to be t h e first w e l l - d o c u m e n t e d case o f u l c e r a t i v e colitis ( U C ) becoming manifest during exacerbations of AP.
CASE REPORT This 21-year-old male student was well until one and one half years prior to admission to the Veterans Administration Research Hospital when he had an episode of bloody diarrhea and abdominal cramping. Six months later he developed two flat erythematous patches on his legs termed "erythema marginatum." Raised purpurie'lesmns appeared on his buttocks, associated with swelling of his left wrist, 8 months prior to admission and 2 weeks after entering the U.S. Army. Subsequently, he had recurrent erythematous eruptions most often on his lower extremities and buttocks, associated with swelling of his hands, feet, ankles, and face. Occasionally, diarrhea and abdominal cramping would accompany the dermatologie and articular symptoms. He was informed that he had ulcerative colitis after one proctoscopic examination while in the mibtary. He was discharged from the Military Service 4 months prior to admission, but the recurrent skin lesions and polyarticular swelling occurred more frequently. One month prior to admission, his private physician placed him on 16 mg methylprednisolone, daily, which partially controlled his dermatologic and articular From the Gastroenterology and Dermatology Sections of the Northwestern University Medical School and the Veterans Administration Research Hospital, Chicago, Illinois. Address for reprint requests: Dr. Robert M. Craig, Gastroenterology Section, VA Hospital, 333 East Huron Street, Chicago, Illinois 60611.
Digestive Diseases, Yo~. 20, No. 5 (May ~g75)
symptoms, and he was gradually tapered to 4 mg daily. He admitted to have had frequent strep throats throughout his life, which had been treated with penicillin. There was no family history of angioneurotic edema, inflammatory bowel disease, or purpura. On his admission physical examination he was in no distress; b~ood pressure was ~34/80 mm Hg, pulse was 88/min and regular, and the temperature was 97.6" F. A l-cm erythematous nodule was present on his right lower leg. There were many hyperpigmented maeules on both legs. There was no lymphadenopathy and his spleen was not palpable. The remainder of his physical examination was normal. The urinalysis, complete blood count, and platelet count were normal on admission, but the hemoglobin fell to 11.0 g/100 ml during the hospitalization. The latex fixation was reactive initially but was negative after 1 week. LE preparations were negative. The ASP titer was 50 Todd units and serum complement was 73 hemolytic units per milliner (normal 40-50). The stools were positive for occult blood on muhiple occasions. Serum chemistries were normal with the exception of an alkaline phosphatase of 125 [U/liter (normal less than 85). The serum protein eleetrophoresis was normal. X-rays of his chest, upper-gastrointestinal tract, lower-gastrointestinal tract, wrists, hands, ankles, and knees were normal. A proctoseopic examination revealed multiple circumscribed erythematous lesions seen throughout the rectal mucosa. There was no friability. A biopsy of the rectum revealed small loci of mucosal hemorrhages and some acute inflammatory cells in the tuniea propria mucosae. A skin biopsy revealed leukoclastic angiitis. During this hospitalization he developed edema of his chest, scrotum, and face with elevated annular lesions on his trunk and extremities. Prednisone 30 mg daily was administered and gradually tapered, and his skin and gastrointestinal symptoms rapidly resolved. A follow-up proetoscopie examination was normal. During the 2 years after this initial hospitalization he had exacerbations of his skin lesions, sometimes associated with watery or bloody diarrhea, so that the dose of prednisone had to be adjusted. During one episode, he required rehospitalization for malaise, cough, occasional hemoptysis, and an associated 1S-pound weight loss, but no gastrointestinal symptoms. The physical examination revealed numerous 1- to 2-mm nonblanching erythematous papules and macules on his legs, buttocks, soles, and palms. His lungs were normal to percussion and auseuhation. The
487
CRAIG ET AL
,Fig 1. Representative area of colonic mucosa demonstrating cryptitis and crypt abscess formation consistent with ulcerative colitis (XlO0).
white blood count was 13,300/mm ~, with a normal differential. The eosinophile count r~anged between 0% and 5%. The hematocrit value was 35%. The platelet count was 365,000. Urinalysis and serum chemistries were normal, except for a persistently elevated alkaline phosphatase, ranging from 180 to 475 IU/liter and a leucine aminopeptidase of 313 Goldbarg-Rutenburg units per 0.02 ml. (normal 75-230). The chest x-ray film revealed bilateral diffuse infiltrates in both lung bases. Immunoelectrophoresis showed IgG, 2000 rag/100 ml (normal 1020 1460); IgA, 330 mg/ 100 ml (normal 210-350); and IgM 83 mg/100 ml (normal 85-155). Countercurrent electrophoresis for hepatitis B antigen was negative. Serum iron was 50 #g/100 ml and the iron binding capacity was 400 ~tg/100 ml. Intermediate strength PPD was negative. The patient was continued on prednisone and was treated for possible tuberculosis with isoniazid, ethambutal, and streptomycin. Subsequently, all sputum cultures were negative for mycobacteria. An oral cholecystogram was normal. A lung biospy was consistent with Loeffler's pneumonitis (The authors are grateful for the consultative opinion of Dr. Averell Liebow who con-
488
firmed the lung biopsy diagnosis). No immune deposits were seen by immunofluorescent techniques. A needle hiospy of the liver was normal. The patient's skin and pulmonary symptoms resolved rapidly, chest x-ray films became normal, and when discharged, isoniazid and prednisone therapy were continued. Over the subsequent year, he had only an occasional erythematous macule. The dose of prednisone was tapered and the patient did not develop diarrhea. Recurrent bloody diarrhea, numerous purpuric lesions on his buttocks, a lowgrade fever, and crampy abdominal pain occurred 15 months later, necessitating readmission to the hospital. On physical examination, temperature was 101 ~ F orally; there Was diffuse abdominal tenderness, with no signs of peritonitis; and scattered purpurie nonblanehing macules over the buttocks and both ankles. The hematoerit was 44 vol%; the WBC was 21,900/mm 3, with increased numbers of band forms. Urinalysis revealed microscopic hematuria but no albuminuria or casts. Creatinine clearance was 120 ml/ rain. The alkaline phosphatase was 300 IU/liter. Stools tested positive for occult blood, but no pathogens were seen
Digestive Diseases, Vol. 20, No. 5 (May 1975)
ULCERATIVE COLITIS AND ANAPHYLACTOID PURPURA
Fig 2. Higher-power magnification of Figure 1 (X 520).
or cultured. The chest x-ray study was normal. A proctoseopic examination revealed a markedly friable, diffuse rectosigmoid mucosa, actively bleeding, and extending beyond 25 cm. A rectal biopsy (Figures 1 and 2) demonstrated an active inflammatory mucosal process with crypt abscesses, consistent with ulcerative colitis. X-ray study of the colon showed loss of haustra in the transverse and descending coIons, and small ulcerations in the descending colon (Figure 3). The patient defervesced, and his skin and gastrointestinal symptoms rapidly resolved with prednisone therapy. He is presently maintained on prednisone as an outpatient. A foBow-up proctoscopic examination appeared normal, and a subsequent rectal mucosal biopsy was interpreted as normal. Digestive Diseases, Vol. 20, No. 5 (May 1975)
DISCUSSION T h e skin h i s t o p a t h o l o g y of a n a p h y l a c t o i d p u r p u r a ( A P ) is a n a r t e r i o l i t i s a n d c a p i l l a r i t i s w i t h e a r l y leukocytoclasis ( n u c l e a r dust) a n d e r y t h r o c y t e e x t r a v a s a t i o n (1). W h e n less active, a less florid, m i x e d c e l l u l a r or m o n o n u c l e a r vasculitis is p r e s e n t . T h e course is self limited in most cases. H o w e v e r , it m a y be r e c u r r e n t or c o n t i n u o u s in a few subjects, p a r t i c u l a r l y in t h e a d u l t (1). T h e histologic a n d clinical s y n d r o m e in t h e a d u l t is s i m i l a r to a n d m e r g e s w i t h hy489
CRAIG ET AL
Fig 3. X-ray of the colon demonstrating loss of haustra and small mucosal irregularities consistent with ulcerative colitis.
490
Digestive Diseases, Vol. 20, No. 5 (May 1975)
ULCERATIVE COLITIS AND ANAPHYLACTOID PURPURA
Fig 4. Representative skin lesions during exacerbation of anaphylactoid purpura.
persensitivity angiitis (2). The present case has typical histopathologic and clinical findings of AP of the recurrent variety. Figure 4 is a photograph of representative lesions during an exacerbation, typical of AP. Of interest was the associated ulcerative colitis found in our patient. Gastrointestinal manifestations of anaphylactoid purpura (AP) have been described frequently in the literature. Early descriptions of AP noted the frequent occurrence of abdominal colic, diarrhea and hematochezia in children (3,4). The relaDigestive Diseases, Vol. 20, No. 5 (May 1975)
tively high incidence of small-intestinal intussusception in this disorder has been described (5). Involvement of the small bowel has been elucidated further in reports on small bowel x-ray films showing localized dilation, or "thumb printing" (6-8). Colonic involvement in AP has been much less frequently described. Although hematochezia and diarrhea are not uncommonly noted in AP, particularly in adults (9), proctoscopic descriptions have been rarely presented, but when described, they were normal or demonstrated only multiple pc491
CRAIG ET AL
techiae (5, 10). There is one report of a patient with AP and a friable mucosa on proctoscopic examination, but no histologic corroboration of ulcerative colitis (UC) was presented (10). This is the first well-documented case the authors have been able to find of ulcerative colitis in an adult with typical AP. T h e diagnosis of ulcerative colitis in this case is substantiated by the diffuse proctosigmoiditis indistinguishable proctoscopically from ulcerative colitis, and the rectal biopsy identical to ulcerative colitis on histologic grounds. There was no evidence for amebic, bacillary, or tuberculous colitis. Further, independent observers, 2 years apart, considered the proctoseopic appearance typical of ulcerative colitis. Finally, the barium enema demonstrated findings consistent with ulcerative colitis. Although not proven by this case report, the occurrence of the two diseases in this patient does not appear to be a fortuitous association, because the skin and gastrointestinal manifestations presented simultaneously during exacerbations of the patient's illness. In addition, both the dermatologic and gastrointestinal aspects of his illness resolved in parallel with prednisone therapy. Although dermatologic disorders are frequently observed in ulcerative colitis, p u r p u r a have been described only rarely (11-14), and no clinical syndrome suggestive of AP has been described in association with ulcerative colitis. In addition to the skin and gastrointestinal aspects of this man's disorders, there were also renal and pulmonary complications. Although focal glomerulonephritis and rapidly progressive glomerulonephritis have been described with AP (10), the usual renal findings are microscopic hematuria, absent cylindruria, and normal renal function, as in this case (9). Biopsy of the' pulmonary infiltrates, demonstrating eosinophilia and vasculitis, is consistent with Loeffler's pneumonitis, and may be a reflection of a hypersensitivity phenomenon underlying the AP syndrome. T h e elevated alkaline phosphatase and leucine aminopeptidase in this patient cannot be readily explained. The 492
oral cholecystogram was normal, arguing against a diagnosis of bilary calculus disease. Unlike the liver biopsies of most patients with abnormal liver function tests and ulcerative colitis, the liver biopsy in the present case was normal, with no apparent pericholangitis (15), although only one portal field was present in the biopsy. Although this is the first well-documented case of ulcerative colitis reported in AP, the association may be a more common occurrence. Careful proctoscopic examinations with rectal biopsies should be performed and reported in patients with AP, especially in those with diarrhea or hematochezia.
SUMMARY
T h e first case of well-documented ulcerative colitis o c c u r r i n g d u r i n g e x a c e r b a t i o n s of anaphylactoid purpura has been presented. T h e patient experienced renal and pulmonary complications. Gastrointestinal manifestations of anaphylactoid purpura were reviewed. It has been advised that other patients with anaphylactoid purpura be carefully observed for evidence of ulcerative colitis.
REFERENCES
1. Wilkinson DS: Henoch-Schoenlein Syndrome, Textbook of Dermatology. Edited by A Rook, DS Wilkinson, and FSG Ebling. FA Davis Co, Philadelphia, 1968, pp 428429 2. Masters R, Herskovic T: Cutaneous manifestations of disorders of the alimentary tract. Dermatology in General Medicine. TB Fitzpatrick, FA Arndt, WH Clark, AZ Eisen, EJ Van Scott, J H Vaughn (eds). New York, McGraw-Hill, 1971, pp 1372-1373 3. Henoch I: Ueber eine eigenthuemliche form von purpura. Berl Klin Wehrschr 11:641-643, 1874 4. Osler W: On the visceral manifestations of the erythema group of skin diseases. Am J Med Sci 127:1-23, 1904 5. Feldt RH, Stikler GB: The gastrointestinal manifestations of anaphylactoid purpura in chilDigestive Diseases, Vol. 20, No. 5 (May 1975)
ULCERATIVE COLITIS AND ANAPHYLACTOID PURPURA
6.
7r.
8,.
9.
10.
dren. Proc Staff Meeting Mayo Clinic 37:465473, 1962 Whitemore WH, Paterson GH: Henoch's purpura: Small intestinal changes. Radiology 46:373-376, 1946 Fetter FS, Mills WL: Roentgenographic findings in Schoenlein-Henoch purpura. A case report. Radiology 55:545-547, 1950 Rodriguez-Erdmann F, Levitan R: Gastrointestinal and roentgenological manifestations of Henoch-Schoenlein purpura. Gastroenterology 260:260-264, 1960 Cream J J, Gumpel J M , Prachey R O G : Schoenlein-Henoch purpura in the adult. Q J Med 39:461484, 1970 Kraemer M: Henoch's purpura. A case with bullous skin lesions and residual scars, roent-
Digestive Diseases, Vol. 20, No. 5 (May 1975)
11.
12. 13.
14. 15.
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