CLINICAL BRIEF

Henoch Schnlein Purpura Presenting as Duodenal Ulcer

and Gastric Outlet Obstruction
Mukesh Rathore & Rimjhim Shrivastava &
Ravinder Goyal & B. D. Radotra & B. R. Thapa
Received: 4 October 2012 / Accepted: 11 March 2013 / Published online: 7 April 2013
#Dr. K C Chaudhuri Foundation 2013
Abstract Henoch-Schnlein purpura (HSP) is an acute
small vessel leucocytoclastic vasculitis. It is the commonest
vasculitis in children, with an incidence of about 10 cases
per 100, 000 a year. Gastrointestinal manifestations are
commonly encountered, however hematemesis and gastric
outlet obstruction are rarely reported. The authors present
the case of a 5-y-old boy having hematemesis, gastric outlet
obstruction and multiple duodenal ulcers. He improved with
steroids and conservative management.
Keywords Henoch-Schnlein purpura
.
Duodenal ulcer
.
Gastric outlet obstruction
.
Endoscopy
Introduction
Henoch Schnlein Purpura (HSP) is the most common child-
hood vasculitis characterized by combination of non-
thrombocytopenic palpable purpura, arthritis or arthralgias, gas-
trointestinal and renal involvement. It is a systemic disease
where antigen-antibody (IgA) complexes activate the alternate
complement pathway, resulting in inflammation and small ves-
sel vasculitis. Gastrointestinal symptoms are present in 5075 %
of patients with HSP [1]. Colicky abdominal pain, vomiting and
gastrointestinal bleeding are the dominant features. Gastrointes-
tinal bleeding is usually occult, but a minority of patients may
have grossly bloody or melenic stools. Duodenum and small
intestine are the most frequently involved sites [2].
The authors report a 5-y-old boy with HSP presenting
with hematemesis and features of gastric outlet obstruction
(GOO) who had large circumferential ulcers in duodenum.
Case Report
A 5-y-old boy presented with epigastric pain abdomen and
non-bilious projectile vomiting for 10 d with hematemesis
for last 4 d to his family physician and was then referred to
the authors. There was no history of non-steroidal anti-
inflammatory drug ingestion, jaundice/hematemesis/arthritis
or arthralgias. On physical examination, patient was pale
and had mild tenderness and fullness with visible peristaltic
movements from left to right in epigastrium. Non-blanchable
macular rashes appeared on extensor aspect of bilateral lower
limbs on day fourteen of illness. Laboratory investigations
showed anemia (Hb 8 g/dL). Rest of the hemogram, liver
and renal function tests, serum electrolytes, serum amylase
and urine analysis were within normal limits. Ultrasound
abdomen showed distended stomach, rugal fold thickening
with slow passage of contents from stomach to duodenum
with possibility of GOO. Upper gastrointestinal endoscopy
(UGIE) showed multiple large circumferential ulcers
extending from the first part of the duodenum to the third part
(D1 to D3). CECTabdomen showed symmetric circumferen-
tial mural thickening involving pylorus with multifocal areas
of mucosal thickening and ulcerations in D1 to D3 along with
segmental mural thickening in jejunal loops. Histopathology
of duodenum showed features of acute duodenitis with
leukocytoclastic vasculitis (Fig. 1) but no evidence of granu-
lomatous pathology. Antral and skin biopsy also showed
features of leukocytoclastic vasculitis. Colonoscopy examina-
tion was normal.
As per European League against Rheumatism & Pediatric
Rheumatology European Society (EULAR/PRES) 2006
criteria [3] and The American College of Rheumatology
M. Rathore
:
R. Shrivastava
:
R. Goyal
:
B. R. Thapa (*)
Division of Pediatric Gastroenterology, Superspeciality of
Gastroenterology, Post-Graduate Institute of Medical
Education & Research, Chandigarh 160012, India
e-mail: brthapa1@yahoo.co.in
B. D. Radotra
Department of Histopathology, Post-Graduate Institute of
Medical Education & Research, Chandigarh, India
Indian J Pediatr (February 2014) 81(2):189190
DOI 10.1007/s12098-013-1010-2
(ACR) Criteria 1990 [4] the patient was diagnosed as having
HSP causing large circumferential duodenal ulcers leading to
GOO. Patient was kept nil per orally initially for 3 d and given
intravenous fluids only. He was treated with oral prednisolone
(2 mg/kg/d) for 2 wk which was tapered thereafter within
2 wk. Symptoms resolved within 1 wk. His diet was advanced
to normal diet at the end of first wk. Endoscopy on day 10 of
admission showed partial healing of duodenal ulcers.
Discussion
Henoch-Schnlein purpura (HSP) is a small vessel
leucocytoclastic vasculitis. Diagnostic criteria include pal-
pable purpura (a mandatory criterion) in the presence of at
least one of the following: (1) diffuse abdominal pain; (2)
any biopsy showing predominant IgA deposition; (3) arthri-
tis or arthralgia; and (4) renal involvement (hematuria
and/or proteinuria) [4] . The mean age of patients is 6 y;
75 % of patients are under 8 y of age and 90 % are less than
10 y of age [5]. The index patient was 5-y-old.
In 1852 % patients gastrointestinal bleed is seen, which
is commonly occult or if overt the commonest symptom is
melena [5]. Gastrointestinal involvements may precede the
cutaneous manifestations in 25 % patients [6] as in the index
patient. The present patient presented with hematemesis
associated with epigastric pain and had melena as well.
Ischemic injury in HSP most commonly involves small
intestine. The D2 is characteristically involved more than
the D1. The UGIE findings commonly described in HSP are
diffuse mucosal redness, small ring-like petechiae and hem-
orrhagic erosions in duodenum [7]. Duodenal ulcers are
comparatively rare. Gastric outlet obstruction due to HSP
is still rarer. The index patient had features of GOO with
multiple large circumferential duodenal ulcers.
Colonic involvement in HSP includes hyperemia, pete-
chiae, ecchymotic lesions resembling the rash, and aphthoid
ulcers [8]. No colorectal involvement was found in index
patient on colonoscopy. Therapy with oral or intravenous
corticosteroids (12 mg/kg/d) is often associated with de-
creased intensity and duration of gastrointestinal symptoms
[9]. In the proband GI symptoms and skin lesion resolved
within one wk of starting steroid along with significant
healing of intestinal lesions. The patient is on regular follow
up in pediatric gut clinic and has gained weight. There has
been no recurrence of symptoms.
To conclude, for patients coming with upper gastrointes-
tinal bleed, the possibility of HSP should also be kept in
mind. Endoscopy is mandatory to define the site, extent and
nature of the lesion and endoscopic biopsies may give clue
to the diagnosis. Steroid therapy is effective for improving
gastrointestinal symptoms as well as for the healing of the
intestinal lesions.
Conflict of Interest None.
Role of Funding Source None.
References
1. Saulsbury FT. Henoch-Schonlein Purpura in children. Report of 100
patients and review of the literature. Medicine (Baltimore).
1999;78:395409.
2. Esaki M, Matsumoto S, Nakamura S, et al. GI involvement in
Henoch Schonlein Purpura. Gastrointest Endosc. 2002;56:9203.
3. Ozen S, Ruperto N, Dillon MJ, et al. EULAR/PRES endorsed
consensus criteria for the classification of childhood vasculitides.
Ann Rheum Dis. 2006;65:93641.
4. Mills JA, Michel BA, Bloch DA, et al. The American College of
Rheumatology 1990 criteria for the classification of Henoch-
Schonlein purpura. Arthr Rheum. 1990;33:111421.
5. Trapani S, Micheli A, Grisolia F, et al. Henoch-Schonlein purpura in
childhood: Epidemiological and clinical analysis of 150 cases over a
5-year period and review of literature. Semin Arthritis Rheum.
2005;35:14353.
6. Chen S-Y, Kong M-S. Gastrointestinal manifestations and compli-
cations of Henoch-Schonlein Purpura. Chang Gung Med J.
2004;27:17581.
7. Tomomasa T, Hsu JY, Itoh K, Kuroume T. Endoscopic findings in
pediatric patients with Henoch-Schonlein purpura and gastrointesti-
nal symptoms. J Pediatr Gastroenterol Nutr. 1987;6:7259.
8. Cappell MS, Gupta AM. Colonic lesions associated with Henoch-
Schonlein purpura. Am J Gastroenterol. 1990;85:11868.
9. Ronkainen J, Koskimies O, Ala-Houhala M, et al. Early prednisone
therapy in Henoch-Schonlein purpura: A randomized, double-blind,
placebo-controlled trial. J Pediatr. 2006;149:2417.
Fig. 1 Duodenal biopsy shows mucosal glands and neutrophilic in-
filtrates in and around vessel (HE-40 X)
190 Indian J Pediatr (February 2014) 81(2):189190