대한소화기학회지 2007;49:114-118
INTRODUCTION
Henoch-Schönlein purpura (HSP), namely anaphylactoid pur- pura, is a distinct systemic vasculitis with IgA dominant immune complex deposits that is characterized by palpable purpura (most commonly distributed over the buttocks and lower extremities), arthralgia, gastrointestinal (GI) signs and symptoms, and glomerulonephrtitis. HSP is more common in children than adults, but it is generally more severe and has more frequent renal involvement in adults. HSP is a rare cause of GI hemorrhage and perforation in adults.
The cause of HSP is unknown, and there are no specific confirmatory tests. Sometimes, GI symptoms precede purpuric rash which makes the diagnosis of HSP difficult, and even may
lead to unnecessary surgical intervention.
We report a 60 year-old man of Henoch-Schönlein purpura with psoas muscle abscess and various GI complications including GI bleeding, ileal microperforation, small bowel obstruction and ileal stricture. Moreover, he developed palpable purpuric skin rashes one month after the admission which made diagnosis of HSP very difficult.
CASE REPORT
A 54 year-old man was hospitalized for the treatment of hypotension, hypoxemia, oliguria associated with abdominal pain and several episodes of vomiting. He was lethargic and severely edematous with a stuporous mentality. He had been
다양한 위장관 합병증과 요근 농양을 보인 He n o c h -S c h ön l e i n 자반증 1 예
건양대학교 의과대학 내과학교실
이희정·김선문·윤성로·이태희·임의혁·허규찬·최용우·강영우
A C a s e o f H e n o c h -S c h ön l e i n P u r p u r a w i t h P s o a s M u s c l e A b s c e s s a n d F u l l -b l o w n G a s t r o i n t e s t i n a l Co m p l i c a t i o n s
H e e J u n g L e e , M . D. , S u n M o o n K i m , M . D. , S u n g R o Y u n , M . D. , T a e H e e L e e , M . D. , E u y i H y e o g I m , M . D. , K y u Ch a n H u h , M . D. , Y o u n g Wo o C h o i , M . D. , a n d Y o u n g Wo o K a n g , M . D.
Department of Internal Medicine, Konyang University College of Medicine, Daejeon, Korea
Henoch-Schönlein purpura (HSP) is a vasculitis involving small vessels of skin, joints, gastrointestinal (GI) tract, and kidneys. The patients typically show palpable purpura with one or more characteristic manifestations including abdominal pain, hematuria or arthritis. HSP shows gastrointestinal symptoms in 50~85% of patients, and in 14~40% of patients GI symptoms precede purpuric rash which makes the diagnosis of HSP difficult. We present a case of Henoch-Sch
önlein purpura with GI bleeding, septic shock by ileal microperforation, small bowel obstruction as a result of ileal stricture and psoas muscle abscess. (Korean J Gastroenterol 2007;49:114-118) Key Words: Henoch-Schönlein purpura; Vasculitis; Psoas muscle abscess
접수: 2006년 5월 23일, 승인: 2006년 11월 29일 연락처: 김선문, 302-718, 대전광역시 서구 가수원동 685
건양대학교 의과대학 내과학교실 소화기내과 Tel: (042) 600-9370, Fax: (042) 600-9090 E-mail: [email protected]
Correspondence to: Sun Moon Kim, M.D.
Department of Internal Medicine, Konyang University College of Medicine, 685, Gasuwon-dong, Seo-gu, Daejeon 302-718, Korea Tel: +82-42-600-9370, Fax: +82-42-600-9090
E-mail: [email protected]
이희정 외 7인. 다양한 위장관 합병증과 요근 농양을 보인 Henoch-Schönlein 1예 115
diagnosed as diabetes mellitus for 1 year, but did not take any hypoglycemic agents. He also had heavy alcohol drinking history for 3 years. His blood pressure was 70/40 mmHg, pulse rate 130 per minute, respiration rate 44 per minute, and body temperature 38.5oC. Oxygen saturation was 65%, under 100 percent oxygen breathing, which led to mechanical ventilator support via an endotracheal tube.
The abdomen was distended and diffusely tender with guarding on palpation over the whole abdomen. Bowel sound was abscent. There was no skin rash. Laboratory tests revealed hemoglobin 10.2 g/dL, white blood cell count 17,900/mm3, C-reactive protein 7.1 mg/dL (normal＜0.375 mg/dL), glucose 274 mg/dL, BUN/Cr 39/3.35 mg/dL, and albumin 2.4 g/dL.
Prothrombin time was 18.2 seconds (normal; 12 to 15 seconds) suggesting septicemia. Urinalysis revealed proteinuria and hematuria. Chest radiographs showed severe pulmonary edema, and abdominal radiographs demonstrated massively dilated small bowel.
Total urine output was only 10 cc for 3 hours. He was in diuretic-unresponsive and fluid overloaded state. Empirical in- travenous antibiotic therapy and continuous renal replacement therapy (CRRT) were performed.
On the second day of hospitalization, we performed upper endoscopy to find the cause of bleeding because 200 cc of fresh blood was drained from Levin's tube. However, there was no evidence of bleeding down to the second portion of the duodenum.
Abdominal and pelvic CT scan revealed nonspecific ileitis and jejunitis, as well as left psoas muscle abscess (Fig. 1). In addition, it suggested the presence of small bowel microperfora-
tion. However, emergency laparotomy was not performed be- cause the perforation was supposed to be sealed with me- sentery. Drainage of the abscess was performed, which was revealed as 300 cc of serosanguineous fluid. Three consecutive blood cultures showed the growth Klebsiella pneumonia, while the abscess fluid culture revealed the growth of Enterococcus faecalis.
On the 17th day of hospitalization, creatinine level reduced from 5.77 to 1.90 mg/dL. Follow-up CT scan revealed an improved psoas abscess with a suspicious focal wall discon- tinuity at the mid ileal loop without ascites and pneum- operitoneum. Small bowel radiography with barium revealed no perforated bowel lesion, which indicated rapid and spontaneous sealing up of the ileal microperforation. We presumed that spontaneous bowel microperforation was due to septic condition initially. His condition improved markedly and that was dis- charged thereafter.
Five days after the discharge, he developed abdominal pain with watery diarrhea and palpable cutaneous purpura on legs (Fig. 2). The abdominal pain was diffuse, constant, dull, nonradiating, which was related with food ingestion. He exhibited voluntary guarding and tenderness by palpation on the left side of his abdomen. The white blood cell count was 9,550/mm3. Stool studies for ova, parasites, bacteria, and Clostrium difficile toxin were negative. Abdominal radiographs showed a massively dilated small bowel (Fig. 3A). Colono- scopy showed an ulcer with peripheral erythema and stricture on the distal ileum, and multiple aphthous ulcers in the sigmoid colon (Fig. 4). The second small bowel study with barium revealed improved segmental wall thickening of the proximal Fig. 1. Abdominal CT findings. (A) An abscess in left psoas muscle (thick arrow) is noted. (B) It shows focal wall discontinuity at mid ileal loop (thin arrow) with perienteric mesenteric fat infiltration.
116 The Korean Journal of Gastroenterology: Vol. 49, No. 2, 2007
jejunum, but aggravated irregular and segmental wall thickening with stricture in the distal ileum (Fig. 3B). Skin biopsy specimens of the leg lesion revealed leukocytoclastic vasculitis suggestive of HSP (Fig. 5A). A terminal ileal biopsy specimen was interpreted as necrotizing small vessel vasculitis infiltrated by polymor- phonuclear leukocytes (Fig. 5B). Kidney biopsy specimens revealed mesangial hypercellularity and mesangial matrix hyperplasia. The glomerular tufts were infiltrated by polymorphonuclear leukocytes.
Immunofluorescent staining showed moderate IgA and C3 deposits in mesangium along the peripheral capillary loops, which was compatible with IgA nephropathy of HSP.
According to the clinical features and histologic findings, the diagnosis of HSP was made. The patient was given oral prednisone 60 mg/day. The patient improved dramatically.
Abdominal pain disappeared within 48 hours followed by disappearance of skin rash.
Fig. 2. Photographic findings of skin. Multiple palpable purpura are noted on both lower legs.
Fig. 3. (A) Supine plain abdomi- nal X-ray. It shows distended small bowel. (B) Barium contrast radiography of small bowel shows markedly narrowed distal ileum (arrow).
Fig. 4. The colonoscopic views show erythematous erosions with stricture in terminal ileum (A) and erythemtous erosion in the cecum (B).
Lee HJ, et al. A Case of Henoch-Schönlein Purpura with Psoas Muscle Abscess and Full-blown Gastrointestinal Complications 117
After four weeks of discharge, creatinine level was reduced from 5.96 to 1.11 mg/dL, and proteinuria from 3.7 to 1.2 g/24 hours. He was discharged with prednisolone which was tapered down to 40 mg/day. Abdominal symptoms subsided later, and the colonoscopy showed healing of an ulcer with mildly improved stricture on the terminal ileum. However, proteinuria and renal insufficiency were continued. Therefore, oral cyclo- phosphamide (200 mg/day) was added to prednisolone, which is now replaced by azathioprine.
DISCUSSION
HSP is a systemic vasculitis syndrome that is characterized by palpable purpura on the skin, arthralgia, GI signs and symptoms, and glomerulonephritis. HSP occurs most frequently in childhood, with a peak incidence of 5 year-old. In adults, it is generally more severe and has more frequent renal involvement.
GI signs and symptoms were reported in 50-85% of HSP patients,1 which varies from mild symptoms to severe com- plications. They included abdominal pain, massive GI bleeding, perforation, intussusception, intestinal obstruction, and infarc- tion.2-5 Abdominal symptoms are caused by submucosal and subserosal hemorrhage, edema within the bowel wall or mesentery, and subsequent thrombosis of the small blood vessels.5,6 Usually purpuric rash appears first but abdominal symptoms may precede typical cutaneous lesions in 14-40% of patients.6 In such clinical circumstance, the pain is sometimes so severe suggesting an acute abdomen that may lead to unnecessary laparotomy. Unnecessary surgery happens in about 10% of patients with HSP.7 GI bleeding occurs in one-third of
the cases, and may present with hematemesis, melena, or bloody stool. Septicemia and small bowel obstruction are rarely reported.8 Small bowel obstruction is believed to be caused by inflammation and necrosis of the blood vessels, resulting in narrowing or occlusion of the lumen. Our patient showed a life threatening course with various GI complications: abdominal pain followed by palpable rashes, GI bleeding, septic shock after ileal microperforation, small bowel obstruction by ileal stricture, and especially psoas muscle abscess which has not been reported in HSP. We thought that the ileal microper- foration resulted in psoas muscle abscess and sepsis, with multiorgan failures such as pulmonary edema, acute renal failure and deteriorated mentality.
Absence of skin manifestations in conjunction with severe GI symptoms is unusual in HSP.9 Chesler et al.9 reported a 5 year-old girl who had suffered from chronic, intermittent abdominal pain for 3 years before skin rash developed. In addition, Kim et al.10 experienced a 34 year-old patient with HSP who died of GI bleeding, massive pulmonary hemorrhage, and respiratory failure. GI symptoms preceded skin manifes- tation for 16 days. These cases have shown that delayed skin manifestation is the major obstacle to an early diagnosis that leads to poor clinical outcome.
The characteristic upper endoscopic findings of HSP are diffuse mucosal redness, small ring-like petechiae and hemo- rrhagic erosions.11The colonoscopy showed aphthous ulcer and hemorrhagic, ulcerating mucosa with marked erythema in the terminal ileum. Our patient showed non-specific finding on upper endoscopy, but multiple aphthous ulcers at the sigmoid colon, erythematous lesion at the appendiceal orifice, and longitudinal ulceration with stricture at the terminal ileum.2,6,12 Fig. 5. Microscopic findings show leukoclastic vasculitis (arrow) with fibrinoid necrosis and inflam- matory cell infiltration of small vessels in skin (H&E, ×100) (A) and in the terminal ileum (H&E,
×400) (B).
118 대한소화기학회지: 제49권 제2호, 2007
Differential diagnosis of these colonoscopic lesions should include Crohn's disease, eosinophilic gastroenteropathy, infec- tious enterocolitis, Behcet's syndrome, and malignancy. Infec- tious origins, such as Yersinia, Campylobacter jejuni, Salmo- nella and Entamoeba historytica were ruled out through appropriate serological and microbiological tests. Other diseases were also ruled out by the biopsy reports and clinical courses.
We considered the possibility of Crohn’s disease until the occurrence of the skin rash and the results of biopsy.
Recently, CT evaluations of the abdomen in patients with HSP have been described. The findings included multifocal areas of bowel wall thickening with skip lesions, mesenteric edema, vascular engorgement, ascites, and nonspecific lymph- adenopathy.13 However, similar small bowel wall thickening may also be seen in eosinophilic gastroenteritis, lymphoma, Crohn’s disease, and granulomatous disease. Our patient showed psoas muscle abscess, nonspecific ileitis and jejunitis, with skip area and focal wall discontinuity at the ileal loop.
The optimal management of GI and renal involvement has not been determined yet.3 Corticosteroids ameliorate joint and GI symptoms in most cases, and improve cutaneous manife- stations in some. However, corticosteroids do not improve the prognosis of HSP with renal involvement.3,14 Some studies suggest that high dose of methylprednisolone, followed by oral prednisone combined with azathioprine or cyclophosphamide may be useful in severe glomerulonephritis associated with HSP.
In summary, we experienced a middle-aged man with HSP, who showed various GI complications. The diagnosis of HSP was very difficult, especially because the abdominal symptoms preceded the cutaneous lesions.
요 약
Henoch-Schönlein purpura (HSP)는 피부, 관절, 위장관과 신장의 소혈관에 염증을 유발하는 질환으로 복통, 혈뇨, 관 절염과 전형적인 피부 자반을 보인다. HSP는 환자의 50- 85%에서 소화기 증상을 보이지만, 14-40%가 자반증보다 소 화기 증상이 앞서 나타나 진단을 어렵게 한다.
이번 증례는 위장관 출혈, 소장의 천공으로 인한 패혈 쇼 크, 소장폐쇄 후 회장 협착과 요근 농양을 보였다.
REFERENCES
1. Smith AI, Serpell JW. Small bowel obstruction in an adult.
Postgrad Med J 1997;73:333-334.
2. Banerjee B, Rashid S, Singh E, Moore J. Endoscopic findings in Henoch-Schonlein purpura. Gastrointest Endosc 1991;37:
569-571.
3. Ozen S. Henoch-Shonlein purpura. Orphanet Encyclopedia 2003;1-3.
4. Shirahama M, Umeno Y, Tomimasu R, et al. The value of colour doppler ultrasonography for small bowel involvement of adult Henoch-Schönlein purpura. Br J Radiol 1998;71:788-791.
5. Lawes D, Wood J. Acute abdomen in Henoch-Schönlein pur- pura. J R Soc Med 2002;95:505-506.
6. Esaki M, Matsumoto T, Nakamura S, et al. GI involvement in Henoch-Schonlein purpura. Gastrointest Endosc 2002;56:920- 923.
7. Karagozian R, Turbide C, Szilagyi A. Henoch-Schönlein pur- pura presenting with ileal involvement in an adult. Dig Dis Sci 2004;49:1722-1726.
8. Robberecht E, Vlaminck H, Gamiz-Jimenez J. Renders F, Van Winckel M, Carton D. Septicemia as a complication of Heno- ch-Schönlein purpura. J Pediatr Gastroenterol Nutr 1997;25:
222-223.
9. Chesler L, Hwang L, Patton W, Heyman MB. Henoch- Schönlein purpura with severe jejunitis and minimal lesions. J Pediatr Gastroenterol Nutr 2000;30:92-95.
10. Kim KJ, Han SY, Yang DK, et al. A case report of Henoch- Schönlein purpura complicated by pulmonary and gastro- intestinal hemorrhage. Korean J Gastroenterol 2000;36:536-541.
11. Ortego-Centeno N, Callejas-Rubio JL, Lopez-Manas JG, Tron- coso-Garcia E, de la Higuera Torres-Puchol J. Ileitis terminalis in a patient with Henoch-Shönlein purpura. Dig Dis Sci 1999;
44:1590-1593.
12. Pore G. GI lesion in Henoch-Schenlein purpura. Gastrointest Endosc 2002;55:283-286.
13. Guanasekaren TS. Henoch-Schönlein purpura. what dose the
“rash” look like in the gastrointestinal mucosa? Pediatr Der- matol 1997;14:437-440.
14. Padeh S, Passwell JH. Successful treatment of chronic Henoch- Shonlein purpura with colchicine and aspirin. Isr Med Asso J 2000;2:482-483.
