Gastrointestinal Endoscopy
Volume 56 • Number 6 • December 2002
Copyright © 2002 American Society for Gastrointestinal Endoscopy
GI involvement in Henoch-Schönlein purpura
Motohiro Esaki MD
Takayuki Matsumoto MD
Shotaro Nakamura MD
Masumi Kawasaki MD
Keiichiro Iwai MD
Katsuya Hirakawa MD
Ken-ichi Tarumi MD
Takashi Yao MD
Mitsuo Iida MD
Received April 1, 2002.
For revision June 5, 2002.
Accepted July 7, 2002.
Current affiliations: Departments of Medicine and Clinical Science and Anatomic Pathology, Graduate School of Medical
Sciences, Kyushu University, Fukuoka, Department of Endoscopic Diagnostics and Therapeutics, Kyushu University
Hospital, Fukuoka, and Division of Gastroenterology, Department of Medicine, Kawasaki Medical School, Okayama, Japan.
Reprint requests: Motohiro Esaki, MD, Department of Medicine and Clinical Science, Graduate School of Medical Sciences,
Kyushu University, Maidashi 3-1-1, Higashi-ku, Fukuoka 812-8582, Japan.
Copyright © 2002 by the American Society for Gastrointestinal Endoscopy
0016-5107/2002/$35.00 + 0 37/1/129592
Background: The diagnosis of Henoch-Schönlein purpura is difficult, especially when
abdominal symptoms precede cutaneous lesions. The aim of this study was to determine the
distribution
of
GI
involvement
in
Henoch-Schönlein
purpura.
Methods: Endoscopic or radiographic findings throughout the entire GI tract were
retrospectively reviewed for 7 patients with Henoch-Schönlein purpura. Histopathologic
findings were analyzed and correlated with findings at EGD and colonoscopy.
Observations: The duodenum and small intestine were most frequently involved (6 patients,
each site). Contrast radiography of the small intestine demonstrated thickened mucosal
folds or small barium flecks. Findings at EGD were multiple irregular ulcers, mucosal
redness and petechiae in the duodenum. In 4 patients, the second part of the duodenum was
predominantly affected. Ulcerating lesions accompanied by hematoma-like protrusions were
detected in 4 patients in whom leukocytoclastic vasculitis was proven histopathologically.
Conclusions: EGD appears to have the greatest diagnostic utility in patients suspected to
have Henoch-Schönlein purpura with GI involvement.
Henoch-Schönlein purpura (HSP) is a vasculitis of the small vessel of the skin, joints, GI tract, and
kidney.[1] [2] GI symptoms occur in up to 85% of patients,[3] and GI involvement can manifest as
severe problems such as intussusception,[4] obstruction,[5] and perforation. [6] Although such severe
manifestations occasionally require surgical intervention, some patients in whom abdominal
symptoms precede the typical skin rash may undergo unnecessary exploratory laparotomy. [7]
Abdominal symptoms reportedly precede other manifestations in 14% of patients. [8] In this clinical
circumstance, GI involvement may be a clue to the diagnosis of HSP.
Mucosal lesions develop anywhere within the GI tract in patients with HSP. Diffuse mucosal
redness, small ring-like petechiae and hemorrhagic erosions are characteristic endoscopic
findings.[9] [11] Radiographically, mucosal thickening and filling defects have been described. [12] [13]
Although the small intestine is considered to be the most frequently affected site, [12] [14] there are no
studies in which the entire GI tract was systematically evaluated in a group of patients with HSP. In
this study, GI involvement by HSP was retrospectively investigated in a group of patients whose GI
tract was thoroughly examined endoscopically or radiographically. Histopathologic findings were
also reviewed to identify endoscopic findings that contribute to the diagnosis of HSP.
Patients and methods
A diagnosis of HSP was made in 16 patients at our institution from 1986 to 2000 based on typical
clinical features including a purpuric rash and histopathologic features in biopsy specimens
obtained from involved skin. In 7 patients, the entire GI tract was endoscopically or radiographically
examined during the acute phase of the disease. These patients were the subjects of the present
investigation. There were 4 men and 3 women whose ages ranged from 13 to 64 years at the time
of diagnosis. In 5 patients, abdominal symptoms occurred within 2 to 10 days after appearance of
the purpuric skin rash. Abdominal symptoms preceded the typical skin rash in the remaining 2
patients (Table 1).
Table 1. Summary of clinical characteristics and GI involvement in patients with HSP
Duodenoscopic findings
Histologic findings
Age
Symptoms
Case (y)/gender at onset
HematomaGI
Mucosal
Irregular like
involvement edema/redness ulcer
protursion LCV
Extravasation
of RBC
ICI
Visceral
involvement Outcome
1
20/M
Epigastralgia, Duodenum,
arthralgia
colon
Moderate
Multiple
Present
Present Marked
Marked
2
52/F
Purpura
Duodenum,
small
intestine,
colon
Mild
Multiple
Present
Present Moderate
Moderate Kidney
Continuing
nephritis
3
13/M
Abdominal
pain,
arthralgia
Stomach,
duodenum,
small
intestine,
colon
Mild
Multiple
None
Absent
Mild
Kidney
Continuing
nephritis
4
64/M
Purpura,
arthralgia
Stomach,
duodenum,
small
intestine,
colon
Moderate
Multiple
Present
Present Marked
Marked
Kidney, lung
Deceased
5
23/F
Purpura
Duodenum,
small
intestine
Marked
Multiple
Present
Present Marked
Marked
None
Improved
6
36/M
Purpura
Duodenum,
small
intestine
Mild
Multiple
None
Absent
Absent
Mild
Kidney
Continuing
nephritis
7
43/F
Purpura
Small
intestine
None
None
None
NE
NE
NE
Kidney
Improved
Mild
None
L
LCV, Leukocytoclastic vasculitis; RBC, red blood cell; ICI, inflammatory cells infiltration; NE, not
examined.
Abdominal symptoms included colicky pain in all patients, nausea and vomiting in 3, and melena in
2. Urinalysis or renal biopsy suggested the presence of nephritis in 5 patients. All patients were
treated with corticosteroids (prednisolone, 40 to 60 mg/day); this was effective in 3, whereas
nephritis persisted in 3 patients. One patient died of pulmonary hemorrhage and renal failure 5
months after diagnosis (Table 1).
Endoscopy and radiography
Improved
GI examinations were performed within 2 to 12 days after the onset of abdominal symptoms.
Preparation for colonoscopy was by ingestion of an oral electrolyte lavage solution. Small intestine
radiography was by either barium meal follow-through or double-contrast technique. For the latter,
200 to 300 mL of 60% barium sulfate and a sufficient volume of air were introduced through a
nasoduodenal tube.
Biopsy findings
At EGD and colonoscopy, biopsy specimens were obtained from the most severe mucosal lesion.
Specimens were embedded in paraffin, cut into slices approximately 4 μm thick, and stained with
hematoxylin and eosin. The small vessel vasculitis with infiltration by polymorphonuclear
leukocytes, referred to as leukocytoclastic vasculitis (LCV), was regarded as the characteristic
pathologic finding of HSP.[15] The following items were assessed for each biopsy by one pathologist
(T.Y.) who was blinded to these clinical features: degree of red cell extravasation and inflammatory
cell infiltration in mucosa and submucosa.
Observations
Endoscopic and radiographic findings
Six patients had involvement of multiple sites within the GI tract (Table 1), the duodenum and small
intestine being most frequently affected.
EGD revealed duodenal mucosal lesions in 6 patients and gastric lesions in 2 patients. The
esophagus was not involved in any patient. Multiple ulcerations in the gastric body or
circumferential redness in the antrum was observed (each in 1 patient). The duodenal mucosal
lesions extended to the second part of the duodenum in 6 patients (Table 1). Involvement in the
second part was more severe than in the bulb in 4 patients.
Although the degrees of mucosal redness, edema, and
petechiae were variable, multiple irregular ulcers were
uniformly found in the duodenum of the 6 patients (Fig. 1).
Fig. 1. Endoscopic view of ulcerating lesion with darkreddish, hematoma-like protrusion in second part of
duodenum (Case 2).
In 4 patients with severe duodenal involvement, ulcers were
accompanied by hematoma-like protrusions.
Small intestinal radiography depicted mucosal abnormalities
in 6 patients. Radiographic findings included thickened
mucosal folds in all 6 patients (Fig. 2) and small barium
flecks in 5.
Fig. 2. Barium contrast radiograph of ileum showing
markedly thickened mucosal folds with large filling defects
(Case
3).
In one patient, there were shallow ulcers in the ileum. Although such findings were widespread in 2
patients, small intestine involvement was restricted to the ileum in 4.
Colonoscopy identified mucosal lesions in 4 patients. In 3, scattered areas of mucosal redness
were found in the rectosigmoid colon. In the fourth patient, there was diffuse mucosal redness and
numerous small ring-like petechiae in the left colon (Fig. 3).
Fig. 3. Colonoscopic view showing diffuse mucosal redness
and small ring-like petechiae in transverse colon (Case 3).
Histopathologic findings
In 4 patients, LCV was identified in duodenal biopsies (Fig. 4).
Fig. 4. Photomicrograph of biopsy from duodenal
lesion showing leukocytoclastic vasculitis with
inflammatory cell infiltration (Case 2) (H&E, orig.
mag.
×25).
Inflammatory cell infiltration and red cell
extravasation in the mucosa and submucosa were
prominent in patients with LCV. All 4 patients had
ulcerating lesions accompanied by hematoma-like
protrusions in the duodenum (Table 1). LCV was
identified in colonic biopsy specimens from one
patient who had diffuse mucosal redness and small
ring-like petechiae. Specimens obtained from the
other 3 patients disclosed nonspecific inflammation
with lymphocytic infiltration.
Discussion
Abdominal symptoms due to HSP include colicky pain, diarrhea, hematochezia, and vomiting. [16] In
one series, exploratory laparotomy was performed because of severe complications in 10% of
patients with abdominal symptoms.[4] Although HSP is less frequent in adults than children, the age
of onset ranges from 6 months to adulthood.[17] Moreover, there have been patients with abdominal
manifestations in whom the typical skin rash was lacking at onset. [8] GI manifestations in the acute
phase of the disease should thus be noted, even in adults.
Although the small intestine is thought to be the most frequent site affected, [12] [14] GI involvement in
HSP has not been systematically investigated to date. In the present study, GI involvement was
analyzed in 7 patients whose GI tract was thoroughly examined during the acute phase of the
disease. The results indicate that the duodenum and small intestine are the most frequently
involved sites.
The duodenoscopic findings described in HSP are diffuse mucosal redness, small ring-like
petechiae and hemorrhagic erosions.[9] [10] [18] [20] In another study, the second part of the duodenum
was found to be predominantly affected. [21] In the present investigation, multiple irregular ulcers
were found in all patients with duodenal involvement. In addition, ulcerations accompanied by
hematoma-like protrusions were identified in 4 patients. However, other duodenoscopic findings,
such as mucosal redness and edema, varied from patient to patient. As has been reported, [21]
duodenal involvement was more prominent in the second part of the duodenum than in the bulb. It
thus seems likely that multiple irregular ulcers in the second part of the duodenum are characteristic
of duodenal involvement in HSP.
Although such duodenal lesions are comparatively rare,[22] eosinophilic enteritis, systemic lupus
erythematosus, Crohn's disease, and parasitic infection are other conditions to be considered in a
differential diagnosis.[23] Biopsies may help to differentiate HSP from these diseases. In the present
study, LCV was found in patients with ulcerating lesions accompanied by hematoma-like
protrusions. Because the frequency of LCV in biopsies correlates with the severity of duodenitis, [14]
and because red blood cell extravasation was prominent in specimens positive for LCV, the
hematoma-like protrusion seems to represent intramucosal hemorrhage due to vasculitis.
Small intestine and colorectal involvement were also detected in our patients. As has been
described,[12] [13] both thickened mucosal folds and small barium flecks noted by small intestine
radiography, and patchy mucosal redness and small ring-like petechiae at colonoscopy were
documented in our patients. However, when considering the preparation required and complexity of
the several procedures, together with accuracy of biopsy findings, duodenoscopy seems to be the
procedure of choice when HSP is suspected in patients with GI symptoms.
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