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Henoch-Schdnlein Purpura: a Diagnosis Not to Be Forgotten Hector Trujillo, MD, T.S

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Henoch-Schdnlein Purpura: a Diagnosis Not to Be Forgotten Hector Trujillo, MD, T.S ■ BRIE f rep o r t Henoch-Schdnlein Purpura: A Diagnosis Not To Be Forgotten Hector Trujillo, MD, T.S. Gunasekaran, MI); Gerald M. Eisenberg, MD; Donald Poiman MD and Ronald Kallen, MD Park Ridge, Illinois Henoch-Schonlein purpura (HSP) is a systemic, generalized vasculitis of unknown etiology thought to be related to an IgA-mediated autoimmune phenomenon. Diagnosis is based on a constellation of physical findings that include the characteristic nonthrombocytopenic petechial or purpuric rash, migratory pol­ yarthralgias, abdominal pain, and renal complications. We report the case of a 19-year-old man with a diagnosis of HSP who had severe abdominal pain and endoscopic documentation of duodenal involve­ ment. Though not clear at presentation, the diagnosis became obvious when the characteristic rash emerged. KEY WORDS. Schonlein-Henoch purpura; abdominal pain; gastrointestinal bleeding; rash. (J Fam Pract 1996; 43:495-498) enoch-Schonlein purpura (HSP) is a langeal joints and tenderness over the left elbow. systemic, generalized vasculitis of All other joints were normal. unknown origin that primarily affects On the first hospital day, several 1-cm red mac­ children but can also occur in adult ules that blanched with pressure were noted over patients. Diagnosis is based on a con­ the ankles. On the third day, these lesions did not stellationH of physical findings, including: (1) a char­ blanch, had increased in number, and were palpa­ acteristic nonthrombocytopenic petechial or pur­ ble. Additional lesions were noted on the back of puric rash, (2) migratory polyarthralgia, (3) renal the legs and the buttocks. The abdomen was involvement, and (4) gastrointestinal involvement. scaphoid with mild guarding, but there was no Failure to recognize this disease may lead to rebound tenderness. Rectal examination revealed unnecessary tests and/or surgery being performed a small amount of stool, which was positive for on these patients. occult blood, but no tenderness or perianal lesions. Laboratory values were: white blood count ■ Case Report (WBC) 11,500/mm', with polymorphonuclear leukocytes 83%, lymphocytes 8%, monocytes 8%, A 19-year-old man presented with a 24-hour history and eosinophils 2%, hemoglobin (Hgb) 15.9 g/dL, of severe joint and muscle pain. He had intermit­ platelets 252,000/mm', erythrocyte sedimentation tent abdominal pain for the previous 2 weeks and a rate (ESR) 24 nun/h, aspartate aminotransferase 12 sore throat for 1 week. The increasing colicky IU/L, alanine aminotransferase 13 IU/L, alkaline abdominal pain resulted in hospitalization. phosphatase 65 U/L, total protein 6.4 g/dL, albumin Physical examination revealed a temperature of 3.5 g/dL, and uric acid 4.6 mg/dL. Rheumatoid fac­ 98°F, weight of 67.6 kg (75th to 90th percentile) tor and anti-nuclear antibody tests were negative. and height of 172 cm (75th to 90th percentile). Urinalysis was within normal limits. Throat culture Examination of the joints showed tenderness and was negative. Plain radiograph of the abdomen swelling of the right elbow and metatarsal pha- was normal. Based on the physical findings and the palpable purpura, the patient’s condition was diag­ Submitted, revised, April 1, 1996. From the Divisions of Pediatric Gastroenterology (H.T., nosed as HSP. TS.G.) and Pediatric Nephrology (R.K.), the Department of To evaluate the gastrointestinal (GI) bleeding Pediatrics, Lutheran General Children’s Hospital, and the and epigastric tenderness, an upper gastrointesti­ Division of Rheuma tology (G.M.E.), Departmen t, of In ternal nal endoscopy was performed. This examination Medicine, avid the Department of Pathology (D.P), Lutheran General Hospital, Park Ridge, Illinois. Requests for reprints revealed extensive ulceration with exudate of the should be addressed to T.S. Gunasekaran, MD, Division of entire descending duodenum and patchy areas of Pediatric Gastroenterology, Lutheran General Children’s redness in the duodenal bulb. The rest of the exam- Hospital, Park Ridge, IL 60068. The .Journal of Family Practice, Vol. 43, No. 5 (Nov), 1996 495 © 1996 Appleton & Lange/ISSN 0094-3509 HENOCH-SCHONLEIN PURPURA ination was normal. Biopsies of the descending diagnosis. In addition, this patient had gastroin­ duodenum showed hemorrhagic and ischemic testinal involvement in the form of severe abdomi­ changes of the surface epithelium and rare fibrin nal pain, hypoalbuminemia, and endoscopically thrombi in the mucosal capillaries. Antral biopsies demonstrated severe duodenal lesions. were normal and silver stain for Helicobacter The reported frequency of gastrointestinal pylori were negative. To evaluate the extent of involvement in HSP varies between 29% and 69%. small bowel involvement, a barium study of the Gastrointestinal symptom s m ost frequently stomach and small bowel was done. This revealed include abdominal pain and guaiac-positive stools. marked irregularity of the descending duodenum Other gastrointestinal manifestations of HSP are with partial narrowing of the third part of the duo­ protein-losing enteropathy,0 functional intestinal denum. No other abnormal findings were noted. obstruction,7 steatorrhea,7’ hydrops of gallbladder,® The diagnosis of HSP was established based on and pseudomembranous colitis.3 Potentially life- the skin lesions and joint and gastrointestinal threatening complications that might need surgery, involvement. The patient was started on 40 mg of such as intussusception, intestinal obstruction, intravenous methylprednisolone. Within 24 hours, bowel infarction, necrosis and perforations, mas­ there was a marked decrease in abdominal pain sive GI bleeding, pancreatitis, hydrops of gallblad­ and an increase in appetite. He was treated for 48 der, and scrotal swelling mimicking testicular tor­ hours with intravenous methylprednisolone and sion. These complications are rare and usually discharged on oral prednisone at 40 mg/day. Within occur less frequently than the 8% reported in one the next 2 weeks, the joint swelling and rash series.8 Increased awareness of these manifesta­ improved. The abdominal pain did not recur. The tions of HSP, particularly abdominal pain or scrotal prednisone was tapered and stopped over 8 weeks. swelling or both in the absence of the typical rash, Follow-up examination at 2 months showed a is needed to prevent unnecessary surgical explo­ weight gain of 3 kg, normal physical examination, ration.8 and stools negative for occult blood. Subsequent Determination of gastrointestinal involvement urinalyses, up to 12 months, were also within nor­ generally occurs through radiological studies,1’ mal limits. The patient refused a repeat upper GI operative observation of intestinal lesions visible endoscopy. from the serosal side,1" or endoscopy.1112 Changes noted from the serosal side of the small bowel dur­ D iscussion ing surgery are similar to those described in inflammatory bowel disease.8 The indications for This case demonstrates several typical features of upper GI endoscopy in patients with HSP are epi­ HSP but also brings to light several atypical fea­ gastric pain, hematemesis, and melena.12 In both tures. HSP is usually considered a disease of chil­ children and adults, there have been few reports of dren: three fourths of patients with this condition endoscopic changes of the gastrointestinal tract are under the age of 7.1 Our patient, at age 19, is associated with HSP.10,1013 Changes described in atypical in that respect. HSP occurs primarily in these reports included gastritis, duodenitis, and males (2:1 ratio), with an incidence of 14 cases per multiple colonic “red” lesions. As in our case, many 100,000 population.1 It occurs most frequently in of the previously reported cases have had severe the spring and fall.23 The disease is preceded by an mucosal involvement of the descending duode­ upper respiratory or pharyngeal infection in up to num, indicating that this site might be a character­ two thirds of the patients.24 Various precipitating istic area of involvement in HSP.12 Similar changes factors, such as streptococcal infection, viral in the duodenum may also be seen in other dis­ agents, insect bites, and drug and food allergies, eases, such as chronic inflammatory bowel dis­ have been implicated as causes of HSP.2 ease. Evidence of vasculitis from the biopsied sam­ Our patient had symptoms that are considered ples combined with the clinical picture, however, classic of HSP: rash, joint involvement, and abdom­ confirms the diagnosis of HSP. H pylon has been inal pain. On initial presentation, however, the rash incriminated as the cause of recurrent HSP in one was atypical in that it blanched with pressure and patient.14 In this case, the skin rash, GI complaints, was macular in appearance, which delayed the and proteinuria improved with eradication of the# 496 The Journal of Family Practice, Vol. 43, No. 5 (Nov), 1996 HENOCH-SCHONLEIN PURPURA pylon. In our patient, H pylon was absent in the involved when intestinal manifestations are antral biopsies. severe. The prognosis is most favorable for those Changes in the GI tract in patients with HSP as with minimal or no renal manifestations at the detected by barium study are nonspecific.14 onset of illness. The mortality cited by Meadow of Changes in the small bowel include separation of 1% to 3% includes patients with severe acute ill­ the loops and mucosal fold thickening. In the ness at onset (rapidly progressive crescentic colon, these changes may appear as mucosal scal­ glomerulonephritis) and those with significant loping and
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