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CASE REPORT Table of Contents   
Year : 2000  |  Volume : 6  |  Issue : 3  |  Page : 165-168
Fatal complication of henoch - schonlein purpura: Case report and literature review


Department of Medicine, King Abdulaziz University Hospital, Jeddah, Saudi Arabia

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Date of Submission30-Jun-1999
Date of Acceptance15-Nov-1999
 

How to cite this article:
Akbar DH. Fatal complication of henoch - schonlein purpura: Case report and literature review. Saudi J Gastroenterol 2000;6:165-8

How to cite this URL:
Akbar DH. Fatal complication of henoch - schonlein purpura: Case report and literature review. Saudi J Gastroenterol [serial online] 2000 [cited 2019 Jun 19];6:165-8. Available from: http://www.saudijgastro.com/text.asp?2000/6/3/165/33481


Henoch Schonlein Purpura (HSP) is a form of Leukocytoclastic vasculitis characterized by abdominal pain, skin rash, arthralgia and hematuria. It is a common vasulitic syndrome of childhood but it is also seen in adults. The syndrome was described by two German physicians in 1837 and 1868 [1] .

The characteristic cutaneous signs of HSP consists of erythematous maculopapular, urticarial lesions with purpura, less often includes hemorrhagic vesicles, bullae and ulcers particularly in adult patients [2],[3] . Gastrointestinal manifestations occur in 35-70% of cases [4],[5] . In most patients, the abdominal symptoms usually subside within a week, even without the use of coricosteroid. About 10% of patients may undergo laparotomy for bleeding, perforation, infarction, intussusception and obstruction [6],[7],[8],[9],[10] . Adults have poorer prognosis than children because of significant increase in the incidence of gastrointestinal and renal dysfunction [11],[12] .

We reported a case of HSP precipitated possibly by Acetyl Salicylic Acid ingestion who presented with hemorrhagic bullae and severe intestinal involvement with subsequent jejunal perforation and death. To the best of our knowledge this is the first report of adult jejunal perforation due to HSP.


   Case Report Top


A 29-years old Ethiopian female presented to our hospital with one week history of generalized skin rash affecting her trunk, upper and lower extremities, knee arthralgia, abdominal pain, vomiting and melena. She was well till 2-weeks prior to admission when she had flu-like illness for which she received 3-tablets of (Acetyl Salicylic Acid 330mg + vit. c 200mg). Three days latter she started developing purpuric rash on her lower limbs which progressed to the abdomen, the back and the upper limbs followed few days latter by generalized crampy abdominal pain associated with vomiting and knee arthralgia. On the day of admission she developed two attacks of melena. There was no fever or urinary symptoms and she had not had other clinically significant history.

On examination her vital signs were stable, skin examination showed hemorrhagic bullae with purpuric macules and papules affecting her extensor surface of the lower limbs. Similar lesions were present on the abdomin, back, buttock and upper extremities [Figure - 1],[Figure - 2]. Targetoid lesions were seen on her abdomen and hands. Knee examination didn't show signs of arthritis or effusion. Cardiopulmonary examination was normal and there was a mild epigastric tenderness on abdominal examination.

On admission, results of the complete blood count, coagulation profile, chemistry tests and urine analysis were normal. Erythrocyte sedimentation rate (ESR) was 17mm/hr. Tests for hepatitis B, C, human immunodeficiency virus I & II serology were all negative. Blood culture as well as antinuclear antibodies, Rheumatoid factor, cryoglobulin, serum C3 & C4 were all negative or normal. Serum IgA was elevated. Chest X-ray, abdominal Ultrasound and ECG were normal. Histological examination of a skin biopsy specimen showed Leucocytoclastic Vasculitis with IgA immune depositis in the wall of the small blood vessels of the dermis. Upper and lower gastrointestinal endoscopy was carried out and it showed diffuse purpuric lesions in the stomach, duodenum, proximal jejunum and the entire colon, with chronic active gastritis and duodenitis. Helicobacter pylori was detected in the gastric mucosa. The patient was started on IN methylprednisolone and a 2-weeks course of omeprazole and amoxacillin. She showed improvements regarding the abdominal pain, disappearance of the melena and improvement of the skin lesions. Ten days latter she had recurrence of the abdominal pain, hematochezia and apperance of new purpuric skin lesions on her lower limbs. A second upper and lower endoscopy was performed. The upper gastrointestinal endoscopy showed the same previous changes but in the lower gastrointestinal endoscopy, colonic mucosa could not be visualized because of the fresh bleeding coming from above. Mesenteric angiography was done to localize the bleeding site for possible resection but it showed diffuse bleeding sites in the jejunum, terminal ileum and the entire descending colon. The patient was switched to pulse steroid therapy with IN methylprednisolone Igm/day for 3 days followed by 80 mg methylprednisolone 8 hourly and blood transfusion to maintain her hemoglobin. The clinical course was complicated by Disseminated Intravascular Coagulation secondary to Klebsilla septicemia. Few days latter the patient had attacks of generalized tonic clonic convulsions. EEG was done and it showed generalized slow waves and C.T of the brain showed generalized brain atrophy which could be due to cerebral vasculitis. As the condition of the patient was not improving she was given a 5-days course of immunoglobulin (0.4gm/kg/d) in addition to the supportive measures with fresh frozen plasma, platelets, antibiotics and tranexamic acid. In spite of all these measures, the patient continued to have hematochezia and one dose of cyclophosphamide Igm IN was given. The patient didn't show much improvement and developed a dynamic ileus and few days latter she developed severe abdominal pain and massive lower gastrointestinal bleeding with abdominal gaurding. Intestinal perforation was suspected, laparotomy was carried out and it showed a giant perforation in the jejunum at 2-meters from the ligament of Trietz. Wedge resection of the perforated bowel was done with end to end anastomosis. Histopathology of the resected segment showed ischemic hemorrhagic necrosis with features of vasculitis. The patient continued to deteriorate and died 4-days latter because of septicemia adult Respiratory Distress Syndrome and multi organ failure.


   Discussion Top


Although HSP is occasionally seen in adults, it is by far more common in children with a peak incidence between 3- to 10- years of age. Onset is usually acute but in 5 - 10% of the patients it may persist or become a recurrent disease. The disease usually resolves completely without sequelae. However, severe involvement of the kidneys and the gastrointestinal tract is responsible for the reported mortality rate of 1-3% [13] . This patient has some forms of the uncommon presentation, precipitating factors and complication. First she gave a history of Acetyl Salicylic Acid (ASA) intake followed 3-days latter by the development of purpuric rash on the lower limbs with hemorrahgic bullae. There has been a report of one case of HSP associated with ASA intake [14] . So, whether ASA alone or in addition to her upper respiratory tract infection was responsible for the precipitation of this severe form of HSP is not known. Second, she presented with hemorrahgic bullae which was described before in HSP [2] and reported in 2- children with no relation between it and the development of complications [15] . It is on note that skin lesions extended to the trunk and the upper extremities yet she didn't develop renal involvement and this was not a predicative factor for it as suggested by Trancrede-Bohin et al [16] . Endoscopy showed diffuse upper and lower gastrointestinal purpuric lesions with gastritis and duodenitis and this has also been reported [17],[18],[19] .

Helicobacter pylori associated with gastritis has been described with HSP and treatment of which resulted in improvement of the skin lesions [20] . But our patient showed only initial response which could be due to the effect of anti-Helicobacter pylori treatment or steroid then she had recurrence. Immunoglobulin has been used in severe intestinal involvement with good response [21],[22] so we tried it in our patient but it didn't help. During her illness she developed convulsions most likely secondary to cerebral vasculitis and this has been reported in children and adults [23],[24] . EEG changes with slow waves had also been described in patients with cerebral vasculities secondary to HSP [25] . Due to the deterioration of the patient condition she was given cyclophosphamide which has been used before in severe intestinal involvement [26] but also it was not of much help. It has been observed that there is no clear evidence of the efficacy of a treatment in complicated cases of HSP [22] . Finally the patient developed jejunal perforation which has been reported only in 2- children [27] . To the best of our knowledge this may be the first case of jejunal perforation due to HSP in an adult. A review of the gastrointestinal complications in the published literatures is shown in [Table - 1].

So in conclusion, although HSP is usually a self limiting disease with good prognosis and outcome, some cases are difficult to manage even with different types of treatment and complications may result in death of the patient particularly in adults.[37]

 
   References Top

1.Schmidt JE, Medical discoveries: who and when. Springfield, III: Thomas 1959;397-8.  Back to cited text no. 1    
2.Piette WW, Stone MS. A cutaneous sign of IgA-associated small dermal vessel leukocytoclastic vasculitis in adults (Henoch-Schonlein Purpura). Arch Dermatol 1989;125:53-6.  Back to cited text no. 2  [PUBMED]  
3.Freedgerg IM, Eiwsen AZ, Wolff K, et al. Fitzpatrick's Dermatology in General Medicine. Fifth edition, volume 11, USA. McGraw-Hill 1999;2048.  Back to cited text no. 3    
4.Rodriquez ER, Levitan R. Gastrointestinal and roentgenological manifestations of Henoch-Schonlein Purpura. Gastroenterology 1968;54:260-4.  Back to cited text no. 4    
5.Cream JJ, Gumpel JM, Peachey RDG. Schonlein-Henoch Purpura in adult: a study of 77 adults with anaphylactoid or Schonlein-Henoch Purpura. Q J Med 1970;39:833-54.  Back to cited text no. 5    
6.Mohamad R. Acute appendicitis: a complication of Henoch-Schonlein Purpura. J R Coll Surg Edinb 1982;27:367.  Back to cited text no. 6    
7.Schwartz S, Boley S, Schultz L, et al. A survey of vascular disease of the small intestine. Semin Roentgenol 1966;1:178-218.  Back to cited text no. 7    
8.Clark CV, Hunter JA. Anaphylactoid Purpura presenting as a medical and surgical emergency. Br Med J 1983;287:22-3.   Back to cited text no. 8    
9.Martinez FLA, Hasse G, Emster JA, et al. Surgical complications in Henoch-Schonlein Purpura. J Ped Surg 1984;19:434-6.  Back to cited text no. 9    
10.Lindenatier SM, Tank ES. Surgical aspects of Henoch­ Schonlein 'purpura. Surgery 1966;982-7.  Back to cited text no. 10    
11.Cream JJ, Gample JM, Peachey RDG. Schonlein-Henoch Purpura in adults. Q J Med 1970;39:461-82.  Back to cited text no. 11    
12.Blanco R, Martinez TV, Garcia FM, et al. Clinical features of Henoch-Schonlein Purpura in young patients and adults (abstract). Arthritis Rheum 1993;(36 suppl):138S(A243).  Back to cited text no. 12    
13.13. Young CP, Krivda MS, Benson LM, et al. Purpuric eruption with bloody diarrhea in adult. Arch-Dermatol 1996;132:1241-4.  Back to cited text no. 13    
14.Alberich RS, Jammoul A, Masana L. Henoch-Schonlein Purpura associated with acetyl salicylic acid. Ann Int Med 1997;26:665.  Back to cited text no. 14    
15.Saulsbury FT. Hemorrahgic bullous lesions in Henoc­Schonlein Purpura described in two children. Pediatr Dermatol 1998;15:357-9.  Back to cited text no. 15  [PUBMED]  [FULLTEXT]
16.Trancrede BE, Ochonisky S, Bignon PMD. Henoch-Schonlein Purpura in adult patients, Predictive factors for IgA glomerulonephritis in a retrospective study of 57 cases. Arch Dermatol 1997;133:438.  Back to cited text no. 16    
17.Cappell MS, Qupta AM. Colonic lesions associated with Henoch-Schonlein Purpura. Am J Gastroenterol 1990;85:1186-8.  Back to cited text no. 17    
18.Mohamad AE, Al Karawi MA. Upper gastrointestinal tract lesions in Henoch-Schonlein Syndrome. Ann Saudi Med 1992;12:499-501.  Back to cited text no. 18    
19.Tung SY, Wu CS, Chen PC, Kuo YC. Clinical observation of Henoch-Schonlein Purpura focus on gastrointestinal manifestation and endoscopic finding. Chang Keng I Hsueh 1994;17:347-51.  Back to cited text no. 19  [PUBMED]  
20.Mozrzymas R, d'Amore ES, Montini G, et al. Schonlein­Henoch vasculitis and chronic helicobacter pylori associated gastritis and duodenal ulcer - a case report. Pediatr Med Chir 1997;19:467-8.  Back to cited text no. 20  [PUBMED]  
21.Hamidou M, Pottier M, Dupas B. Intravenous immunoglobulin in Henoch-Schonlein Purpura. Ann Int Med 1996;125:1013.  Back to cited text no. 21    
22.Ruellan A, et al. Rheumatoid purpura and intravenous immunoglobulins. Rev Med Intern 1997;18:727-9.  Back to cited text no. 22    
23.Ha TS, Cha SH, Cerebral vasculitis in Henoch-Schonlein Purpura: a case report with sequential magnetic resonance imaging. PediatrNephrol 1996;10:634-6.  Back to cited text no. 23    
24.Fielding RE, Hawkins CP, Hand MF, et al. Seizures complicating adult Henoch-Schonlein Purpura Nephrol Dial Transplant 1998;13:761-2.  Back to cited text no. 24    
25.Ostergaard JR, Storm K. Neurological manifestations of Henoch-Schonlein Purpura. Acta Pediatr Scand 1991;80:339-42.  Back to cited text no. 25    
26.Kamm M, John S, Riess R, et al. The first manifestation of Henoch-Schonlein Purpura in a 74-years old patient with hyperthyroidism. Dtsch Med Wochenschr 1997;122:54-8.  Back to cited text no. 26  [PUBMED]  
27.Cull DL, Rosario V, Lally KP, et al. Surgical implications of Henoch-Schonlein Purpura J Pediatr Surg 1990;25:741-3.  Back to cited text no. 27    
28.Choong CK, et al. Colo-colic intussusception in Henoch­Schonlein Purpura. Pediatr Surg Int 1998;14:173-4.  Back to cited text no. 28  [PUBMED]  [FULLTEXT]
29.Chao SC, Huang JG. Ilea] perforation in Henoch-Schonlein Purpura report of one case. Chung-Hua-Miu-Kuo-Hsia-Erh­Ko-I-Hsueh-Hui-Tsa-Chih 1996;37:455-7.  Back to cited text no. 29    
30.Weber TR, et al. Massive gastric hemorrhage: an unusual complication of Henoch-Schonlein Purpura. J Pediatr Surg 1983;18:576-8.  Back to cited text no. 30  [PUBMED]  [FULLTEXT]
31.Takamatsu K, et al. Henoch-Schonlein Purpura with rapidly progressive glomerulonephritis and fatal intraperitoneal hemorrhage in an adult. Nippon Jinzo Gakkai Shi 1994;36:63-8.  Back to cited text no. 31  [PUBMED]  
32.Santiago J, et al. Henoch-Schonlein Purpura with hemnorrhagic ascites and intestinal serositis. Gastrointest Endosc 1996;31:809-11.  Back to cited text no. 32    
33.Gow KW, et al. Multiple entero-enterol fistula: an unusual complication of Henoch-Schonlein Purpura. J Pediatr Surg 1996;31:809-11.  Back to cited text no. 33  [PUBMED]  [FULLTEXT]
34.Van WPM, et al. Esophageal stricture as a complication in Henoch-Schonlein Purpura Eur J Pediatr Surg 1992;2:236-8.  Back to cited text no. 34    
35.Lombard KA, et al. Ileal stricture as a late complication of Henoch-Schonlein Purpura. Pediatrics 1986;77:396-8.  Back to cited text no. 35  [PUBMED]  
36.Levy WFE, et al. Acute pancreatitis in rheumatoid purpura. A propos of 2-cases. Rev Med Interne 1997;18:54-8.  Back to cited text no. 36    
37.McCrindle BW, Wood RA, Nussbaum AR. Henoch­Schonlein Syndrome: unusual manifestations with hydrops of the gallbladder. Clin Pediatr (phila) 1988;27:254-6.  Back to cited text no. 37  [PUBMED]  

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Correspondence Address:
Daad H Akbar
Department of Medicine, King Abdulaziz University Hospital, P.O. Box 18298, Jeddah 21415
Saudi Arabia
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PMID: 19864714

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