0002-9270/86/8003-0195Tut AMERICAN JOURNAL OF GA.STROLNTE;ROLO(;y

Copyright© 1986 by Am. Coll. ofGasiroenterologyVol. 81. No. 3. 1986

Printed in U.S.A.

Leucocytoclastic Vasculitis (Hypersensitivity Angiitis) of theSmall Bowel Presenting with Severe Gastrointestinal

Hemorrhage

Farooq P. Agha, M.D., F.A.C.G., Timothy T. Nostrant, M.D., and David F. Keren, M.D.Department oj Radiology, Internal Medicine-Division oj Gastroenterology. and Pathology. University oj Michigan Hospitals and

Medical Center, Ann Arbor, Michigan

A case of leucocytoclastic vasculitis involving theentire small bowel is reported. A high index of suspicionand recognition of the early palpable purpuric skinlesions in patients with acute abdominal pain and gas-trointestinal hemorrhage might avert unnecessary sur-gical exploration in some patients.

INTRODUCTION

Leucocytoclastic vasculitis (LCV) and Henoch-Schonlein purpura (HSP) are syndromes characterizedby vasculitis ofthe postcapiUary venules and "palpablepurpura" as the clinical hallmark (1-5). Gastrointes-tinal involvement associated with vasculitis has beenwell recognized in children with HSP (7-11) and inpatients with polyarteritis nodosa (12-14), but infor-mation regarding diagnosis and management of leuco-cytoclastic vasculitis with gastrointestinal involvementis limited to few reports (I, 15-17). This report illus-trates a case of LCV with severe gastrointestinal hem-orrhage and emphasizes the systemic nature of thisdisease and urges restraint before performing explora-tory surgery on such patients.

CASE REPORT

A 35-yr-oId white man sustained 10% bums of hisright upper extremity after a firework accident on July4, 1984. He underwent partial and full thickness skingrafts at a local hospital. He was recovering well untilJuly 24th. when he developed crampy abdominal painassociated with nausea., vomiting, and passage of mela-notic stools per rectum. He was transferred to theUniversity of Michigan Hospitals for management.

His past medical history was remarkable for hepatitis-A in 1968 and traumatic partial amputation ofthe leftfifth finger. There was no history of intravenous drugabuse or previous blood transfusions.

At the time of admission to the University Hospitalhis vital signs were within normal limits except for aresting tachycardia of n6/min without orthostaticchanges. The physical examination including ausculta-

tion of the chest was unremarkable. The abdominalexamination revealed mild periumbilical tendernesswithout rebound or rigidity. The rectal examinationrevealed melena and no other abnormalities.

The admission laboratory findings revealed Hb of16.6 g. hematocrit 50.4%. white blood cell count of24,300/mm^ with a slight left shift, and normal plateletcount. The blood urea nitrogen was 11, creatinine 0.9,and the serum electrolytes were normal. The proteinelectrophoresis showed mild hypogammaglobulinemiaand antinuclear antibody screening was negative. Hisurinalysis showed no evidence of proteinuria.

Plain abdominal radiographs revealed an abnormalloop of small bowel in the left side of the abdomenshowing thickened mucosa and thumbprinting (Fig.Iv4), scattered gas in the small bowel, and minimaldistension of the transverse colon. Upper endoscopyshowed no significant abnormality in the esophagus,stomach, and duodenum. Flexible sigmoidoscopy wasnormal up to 40 cm. A small bowel follow throughexamination revealed marked submucosal edema ofthe jejunum and proximal small bowel (Fig. \B). Theabdominal pain persisted with intermittent melanoticstools. Visceral angiography was performed on the 3rdday after admission which showed normal mesentericvasculature. Twenty-four hours after angiography hisabdominal pain became constant and increased in in-tensity. Repeat stool examination revealed bright redblood. The skin examination at ihis time revealed twopetechiae and purpuric spots on his lower extremities.His Hb dropped to 10.5 g and the hematocrit to 48%.The white blood count increased from 24,300/mm-^ to38.700/mm^ Bowel ischemia with possible infarctionwas suspected and the patient underwent exploratorylaparotomy on the 4th day after admission. At surgerythe entire small bowel was inflammed with numerousscattered patches of petechia! hemorrhages on the se-rosal surface. These changes were more severe in thejejunum and proximal small bowel. No mechanicalobstruction or mass lesion was found. The colon wasnormal and the remainder of the abdominal explora-tion was unremarkable. The bowel was viable and no

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FIG. I. .-1, plain abdominal radiograph shows a Ihickened edematous loop of small bowel in the left upper quadrant wilh "thumbprinting"indicating sumucosal infiltration {arrow.s). B. a small bowel examination shows mucosal thickening and edematous loops of jejunum andproximal small bowel wilh no evidence of obstruction or mass lesion.

resection was done. Biopsies of the small bowel andpurpuric lesions from his lower extremity were ob-tained. He was treated with intravenous hydrocortisoneintraoperatively and postoperatively. The histopatho-logica! examination of the biopsied material revealedLCV affecting the small bowel and the skin lesions (Fig.2). Direct immunofluorescence examination ofthe pur-puric skin lesions and the bowel using monospecific-fluorescein isothiocynate-conjugated antihuman IgG,IgA, IgM, C3, and fibrin showed weak granular IgAdeposition in the small vessels along with intense fibrindeposition. There was no deposition of IgG, IgM, orC3. The fmdings were consistent with immune-com-plex vasculitis (leucocytoclastic). He was treated withsteroids which were gradually tapered and he was dis-charged on the 12th day of hospitalization. At 1-yrfollow-up there is no recurrence of vasculitis,

DISCUSSION

LCV is a pathological designation characterizingthose conditions in which the blood vessel walls areinfiltrated by polymorphonuclear leucocytes and nu-clear fragments in varying stages of necrosis (I). Thepostcapillary venule is the usual site for this lesion (2).There is reasonable evidence that this inflammatoryprocess is initiated by the subendothelial deposition ofantigen-antibody complexes which are derived from the

circulation. These immune complexes activate thecomplement system, releasing chemotactic factorswhich attract polymorphonuclear leucocytes. Proteo-lytic enzymes are then released and mediate the vas-cular necrosis (3. 4).

LCV or necrotizing vasculitis consists of several dis-tinct clinical syndromes affecting small vessels less thanO.I mm in diameter, which are characterized by im-munecomplex deposition of IgA in the walls of theaffected vessels and some form of complement activa-tion. Hypersensitivity angiitis, allergic vasculitis, cuta-neous systemic vasculitis, and hemorrhagic capillarytoxicosis are other terms used for this syndrome com-plex. Identical pathological changes are seen in anaphy-Iactoid or Henoch-Schonlein purpura, hypocomple-mentemic vasculitis, and essential mixed cryoglobuli-nemia (4). Skin involvement is very common and isoften the only site affected by characteristic leucocyto-clastic vasculitis. The skin lesions first appear on thelower extremities as flat erythematous or urticaria!patches progressing to purpuric papules and are mostlyconcentrated on dependent areas. The palpable qualityof these lesions is an important feature since this distin-guishes them from other forms of purpura (4). In ad-dition to the characteristic purpuric papules, hemor-rhagic bullae and irregular superficial erosions coveredby an eschar may be seen. The disease may be limitedto the skin or there may be systemic involvement of

March 1986 LEUCOCYTOCLASTIC VASCULITIS 197

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Fici. 2. .-f. this photomicrograph ot the small bowel biopsy from the same patient demonstrates passing through the muscularis mucosa {M)a vessel (!') which in the submucosa becomes distorted {arrows) by acute inflammation with prominent fibrin deposition (/•') (hematoxylin andeosin. original magnification X470}. B. this photomicrograph ofthe skin biopsy from the present case discloses a classic leukocytoclastic vasculitiswith a vessel {arrow) being infiltrated by acute inflammatory cells and showing prominent endothelial proliferation that obscures the lumen(hematoxylin and eosin original, magnification x470).

joints, kidneys, lungs, and the gastrointestinal tract.This type of vasculitis may be precipitated by a drug ora microorganism, but often the specific antigen whichtriggers the process remains unidentified.

Lopez et al. (5) in a review of 93 cases with LCVreported that 32 (34%) had significant gastrointestinalmanifestations. They further noted that gastrointestinalmanifestations were much more common in patientsyounger than 16 yr of age (66%) than in older patients(26%). Gastrointestinal tract involvement by LCV maymanifest as abdominal pain that is usually colicky innature. Nausea, vomiting, and diarrhea are often pres-ent. Mild to moderate gastrointestinal bleeding has beenreported in 52% ofthe cases and 21% ofpatients maypresent with acute abdomen (5).

HSP is established as a distinct subtype of LCV. Itmay involve any age group, but the majority of patientsare children with a peak incidence from 4 to 8 yr of age{8-11), Although the small bowel is more frequentlyinvolved, cases of esophageal, gastroduodenal, and co-

lorectal involvement has been rarely reported in HSP,Abdominal complications such as intussusception,bowel obstruction, perforation and ischemic necrosishave been reported in patients with HSP. Althoughgastrointestinal tract involvement is found in over 50%of children with HSP, only few reports describe thegastrointestinal manifestations associated with LCV orHSP in adults. Information regarding diagnosis andmedica] management of gastrointestinal vasculitis syn-dromes in adults is thus limited (1, 7, 15, 16).

The demonstration of weak granular IgA and intensefibrin deposition in the small vessels in our patient isconsistent with an immunecomplex vasculitis (4-6).The source of this antigenic stimulation is unknown.These patients usually present difficult diagnostic di-lemma. The clinical history is not helpful at all. Bariumstudies of the gastrointestinal tract may show a spec-trum of nonspecific features ranging from mucosaledema to typical thumbprinting. Occasionally radio-graphic changes may simulate Crohn's disease or acute

198 AGHA et al. Vol. 81, No. 3, 1986

ileitis. Endoscopy ofthe upper or lower intestinal tractmay reveal submucosa! hemorrhage with or withoutfocal vasculitis. Recently computed tomographic ap-pearance of segmental mural thickening and luminalnarrowing in LCV correlating well with the bariumsmall bowel studies and endoscopic fmdings have beenreported (18). Endoscopic biopsies may reveal mucosalnecrosis or small vessel involvement in the laminapropria. Although the temporal association of palpablepurpura to gastrointestinal manifestations in LCV isvariable, it is advisable that adults with gastrointestinalbleeding without obvious source be examined for pal-pable purpura of LCV. This may detect potential casesof LCV and would allow steroid therapy to produceresolution of symptoms and thereby avoiding unnec-essary surgery.

ACKNOWLEDGMENT

The authors thank Ms. Barbara Smith for excellentsecretarial assistance.

Reprint requests: Dr. Farooq P. Agha. Department of Radiology-Box 013. University Hospital. 1405 East Ann Street. Ann Arbor. M!48109.

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3. Sams WM. Claman HN. Kohler DF. et al. Human necrotizing

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4. Gilliam HN. Smiley JD. Cutaneous necrotizing vasculitis andrelated disorders. Ann Allergy 1976:37:328-39.

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