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| Year : 2000 | Volume
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| Issue : 3 | Page : 165-168 |
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| Fatal complication of henoch - schonlein purpura: Case report and literature review |
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Daad H Akbar
Department of Medicine, King Abdulaziz University Hospital, Jeddah, Saudi Arabia
Click here for correspondence address and email
| Date of Submission | 30-Jun-1999 |
| Date of Acceptance | 15-Nov-1999 |
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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 |
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 | |  |
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 | | |
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]
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Correspondence Address:
Daad H Akbar
Department of Medicine, King Abdulaziz University Hospital, P.O. Box 18298, Jeddah 21415
Saudi Arabia
 Source of Support: None, Conflict of Interest: None  | Check |
PMID: 19864714 
[Figure - 1], [Figure - 2]
[Table - 1] |
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