ORIGINAL ARTICLES

Recurrent Henoch-Schonlein Purpura in Children

Dario Prais, MD, Jacob Amir, MD, and Moshe Nussinovitch, MD

Background: Henoch-Schonlein purpura (HSP), also known as

anaphylactoid purpura is a clinically recognizable systemic disorder
occurring in children, mainly from ages 3 to 10 years.
Objectives: To describe the clinical, epidemiological, and laboratory findings in a group of patients with recurrent HSP, admitted to
a tertiary pediatric center.
Methods: Retrospective analysis of medical records of patients
hospitalized due to HSP between 1969 and 2004.
Results: Two hundred sixty children (56.7% males) were hospitalized due to HSP, 7 (2.7%) more than once. There were no statistically significant differences in demographic or clinical characteristics between the patients with 1 event of HSP and patients with
recurrence. Mean age of the subgroup with recurrence was 3.67
years (10 months to 7.4 years) at the first episode, and 5.03 years
(2.210 years) at the second one, with a mean lag period of 13.5
2.8 months (range 226). The duration of the recurrent clinical
symptoms ranged from 9 to 30 days, and in 72% of those patients,
resolution took more than 14 days.
Conclusion: In our inpatient population, no clinical or laboratory
characteristics were found to be predictive of recurrence; the second
episode was longer than the first and the lag period between the 2
episodes was substantially longer than previously reported. Hospital
admissions for recurrent HSP are not common. Nevertheless, a good
prognosis was the rule of our admitted patients.
Key Words: vasculitis, anaphylactoid, recurrence, prognosis,
Henoch-Schonlein
(J Clin Rheumatol 2007;13: 2528)

enoch-Schonlein purpura (HSP), also known as anaphylactoid purpura is a clinically recognizable systemic
disorder occurring in children, mainly from ages 3 to 10 years
and in young adults.1 It is characterized by a distinctive
purpuric rash distributed symmetrically over the buttocks and
lower extremities, but sometimes involving the upper extremities, trunk, and face. Systemic symptoms and signs include
edema (particularly in the younger age group),2 joint involvement (arthralgia or arthritis), gastrointestinal manifestations
From the Department of Pediatrics C, Schneider Childrens Medical Center
of Israel, Petah Tiqva and Sackler Faculty of Medicine, Tel Aviv
University, Tel Aviv, Israel.
Reprints: D. Prais, MD, Department of Pediatrics C, Schneider Childrens
Medical Center of Israel, 49202 Petah Tiqva, Israel. E-mail: dariop@
clalit.org.il
Copyright 2007 by Lippincott Williams & Wilkins
ISSN: 1076-1608/07/1301-0025
DOI: 10.1097/01.rhu.0000255692.46165.19

(abdominal pain, blood diarrhea), and renal damage (hematuria, nephritis). In rare cases, there may be central nervous
system, scrotal, pulmonary, or cardiac complications. Diagnosis is usually made clinically. Skin biopsy, which typically
shows leukocytoclastic vasculitis, is generally unnecessary.
The underlying pathologic process of HSP is an immunecomplex-mediated inflammation of the small vessels.3 The
site, extent, and severity of vessel involvement in the individual patient determine the clinical picture. Although the
nature of the immunologic reaction is unclear, the frequent
reports of upper respiratory tract infection or streptococcal
pharyngitis preceding the onset of symptoms suggest a hypersensitivity phenomenon.
There are several reports in the literature of long-term,
recurrent HSP. Most concern the recurrence of renal impairment. Full-blown recurrent HSP, with the characteristic clinical presentation including the distinctive purpuric rash, has
been described in only a few cases.4 Its incidence in the
pediatric population is unknown.
The aim of the present study was to report the clinical,
epidemiological, and laboratory findings of patients with
recurrent HSP admitted to a tertiary pediatric center during
the past 30 years.

PATIENTS AND METHODS

The medical records of all children discharged from our
tertiary pediatric medical center with a diagnosis of HSP from
1969 to 2004 were analyzed. The diagnosis was confirmed by
clinical, laboratory, and in some cases, pathology studies.
Inclusion criteria were presence of nonthrombocytopenic palpable purpura with or without systemic involvement, characteristic purpuric rash, and absence of any other disease or
medication known to cause purpura. Since the diagnosis of
HSP is mainly clinical, patients who did not undergo skin
biopsy were not excluded; however, patients had to fulfill the
American College of Rheumatologys criteria for diagnosis
of HSP.5 Demographic data associated clinical manifestations, and laboratory, radiologic, and pathologic findings
were summarized.
Patients diagnosed more than once with HSP were
classified as having recurrent HSP. The inclusion criteria for
this subgroup were the same as for all patients with HSP, in
addition to the reappearance of the characteristic purpuric
rash (with or without associated symptoms), following a
previous complete remission. The medical records of the
patients with recurrent disease were rechecked to determine
that diagnosis was confirmed. The patients were followed in
the outpatient clinic or by telephone. Data were analyzed for

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Prais et al

statistical significance with the Fisher exact test and Student

t test.

On long-term follow-up (at least 2 years), no further

recurrent episodes were reported, and no complications or
persistent symptoms were found.

RESULTS
During the study period, 260 children were hospitalized
with HSP; 56.7% males and 43.3% females; mean age at
presentation was 5.7 2.6 years (range 114.8 years). Mean
duration of the skin involvement was 11.5 days (range 327
days); 12% of patients showed skin involvement for more
than 15 days. Systemic involvement was arthritis and arthralgia in 68%, abdominal pain in 59%, hematuria or proteinuria
in 35%, and impaired renal function or acute renal failure in
3%. Documented evidence of a previous bacterial or viral
infection was found in 12% of patients; 8 patients had
positive antistreptolysin O test (of the 193 patients tested,
4%). Immune-globulin A levels were elevated in 2 of the 6
examined patients.

Recurrent HSP
Seven of the 260 patients were hospitalized more than
once for HSP (2.7%). Their clinical and epidemiological
characteristics are presented in Table 1. There were no
statistically significant differences between the entire patient
group and the recurrence subgroup in sex or age distribution,
clinical presentation, systemic involvement, duration of
symptoms, and need for medical treatment during the first
episode (P 0.1, data not shown). All patients reported a
nonspecific viral illness during the month before presentation.
Forty-three percent of the recurrence subgroup were
male. Mean age at the first episode was 3.67 2.08 years
(range 10 months to 7.4 years); at the second episode, 5.03
2.6 years (range 2.210 years). Mean lag period between the
2 episodes was 13.5 2.8 months (range 226 months).
During the second episode, all patients had the characteristic cutaneous involvement and abdominal pain of differing severity at various stages of the disease. Arthralgia was
noted in 5/7, hematuria in 3/7, proteinuria in 2/7, bloody
stools and vomiting in 1 patient each. Laboratory studies
were not diagnostic: mean leukocyte count was 9800 800
cells/mm3 (range 8300 12,200); C-reactive protein level,
erythrocyte sedimentation rate, immunoglobulin levels, and
coagulation test were within normal range. Normal findings
were found on urinalysis, except for 2 cases of microhematuria and mild proteinuria (150 mg/24 hours), and on stool
examination for occult blood, except for 1 case of overt
bloody diarrhea. No cases of severe renal impairment were
seen. Other serological tests (antinuclear antibody, antineutrophilic cytoplasmic antibody, anticardiolipin) were negative. Antistreptolysin O was positive in 1 child.
Because of the recurrent nature of the episodes and the
need to confirm the diagnosis, skin biopsy was performed in
5 patients. All showed leukocytoclastic vasculitis. Immunoglobulin A deposits were present in 4 patients, and C3
deposits in 1. Corticosteroid treatment (2 mg/kg/d) was prescribed in 2 patients: 1 with bloody diarrhea and the other
with severe abdominal pain.
The duration of the clinical symptoms ranged from 9 to
30 days (mean 17.85 days); in 72% of the patients, resolution
took 15 days or more.

26

DISCUSSION
HSP, the most common acute vasculitis affecting children, is usually clinically diagnosed on the basis of the classic
triad of nonthrombocytopenic purpura, abdominal symptoms
(Henoch), and joint complaints (Schonlein). Full-blown
recurrent HSP, with the characteristic clinical presentation including the distinctive purpuric rash, was previously
reported,4 but the incidence in the pediatric population remains
unknown.
We describe 7 cases of recurrent HSP requiring rehospitalization in our pediatric population. All patients had the
characteristic purpuric rash and abdominal pain. Arthralgia,
renal involvement, and gastrointestinal bleeding were less
frequent. Full recovery was the rule, with no sequelae.
Most of the few published reports of recurring HSP
cases described unusual or atypical presentations, such as
Wiskott-Aldrich syndrome in an 8-year-old boy6; abnormally long intervals between onset of abdominal pain and
skin rash (24 weeks) in a 7-year-old boy7; tube-ovarian
abscesses in a 15-year-old girl8; recurrent testicular swelling
and orchitis in a 4-year-old boy.9 Nathawani et al4 described
5 patients with recurrent HSP, including 2 children: a 16year-old who experienced the second episode 7 years after the
first, and a 3-year-old with a recurrent episode of nephritis
following a throat infection.
Recurrence of HSP commonly occurs within 23
months of the primary episode. The incidence may be higher
than expected, since some cases of recurrence may be misdiagnosed as part of a prolonged course of primary HSP.
Saulsbury1 reported a 33% incidence of recurrence, defined
as reappearance of the characteristic rash or other symptoms
following resolution of the disease for at least 2 weeks. In 16
of their patients (48%), recurrence occurred during the first
month, and only 2 had a recurrence beyond 4 months.
Similarly, Trapani et al reported a recurrence of purpura in
35% of patients, occurring within 12 months after the acute
phase of the disease.10 When comparing this to our data, it
must be emphasized that we included only rehospitalized
patients.
In general, the recurrent episode tended to mimic the
original one. Our population is unique in 2 aspects: the lag
period between the 2 episodes of HSP was substantially
longer than in other reports (mean 13.5 2.8 months), and
the second episode (especially the skin rash) tended to be
longer than the first. However, in our series, similar to
previous studies, there were no significant differences in the
clinical presentation between the first and second episodes.
The incidence of systemic involvement was also comparable.
There were also no significant differences in the clinical
presentation of the first episode between the entire group of
patients and the subgroup with recurrent HSP. Thus, no
specific characteristics of the first episode could be identified
that would predict patients at risk for recurrences. As in other
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 2007 Lippincott Williams & Wilkins

F
M
M

3
4
5

3.67*
2.08

2.6
3.5
7.4

2.5

0.8

Age
(yr)

Streptococcal
pharyngitis

Viral

Viral

Viral

Previous
Infection

Arthritis

AP, renal

AP, renal,
arthritis
AP, renal,
arthritis
AP
AP
AP, renal,
arthritis

Systemic
Symptoms

AP, abdominal pain; URTI, upper respiratory tract infection.

*P 0.005 between 1st and 2nd episode.

Mean
SD

Sex

Patient
No.

9.14*
4.1

13

12

5
7
15

Duration
Symptoms (d)

First Episode

No

Yes

No
Yes
Yes

No

No

Skin
Biopsy

Supportive

Steroids

Supportive
Supportive
Steroids

Supportive

Supportive

Treatment

TABLE 1. Clinical and Epidemiological Characteristics of Patients with Recurrent HSP

5.03*
2.6

5.5

2.8
5
10

3.75

2.2

Age
(yr)

Pharyngitis

Viral
Viral
URTI

Viral

URTI

Previous
Infection

AP, hematuria
AP, arthralgia
AP, arthritis,
proteinuria,
bloody stools
AP, arthralgia,
microhematuria
Arthralgia,
microhematuria,
proteinuria

AP

AP, arthritis

Systemic
Symptoms

17.85*
7.7

20

9
24
30

18

15

Duration
Symptoms (d)

Second Episode

No

Yes

No
Yes
Yes

Yes

Yes

Skin
Biopsy

Supportive

Supportive

Supportive
Supportive
Steroids

Steroids

Supportive

Treatment

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Recurrent Henoch-Schonlein Purpura in Children

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JCR: Journal of Clinical Rheumatology Volume 13, Number 1, February 2007

Prais et al

studies, our patients, including those with recurrent diseases,

had a good prognosis.1,7,9,10
Our lower incidence of recurrence compared with previous reports may be explained by the longer lag period
between episodes in our population. Another explanation
may be that our sample was limited to patients hospitalized in
a tertiary pediatric medical center.
This report should shed further light on the rarely
reported clinical and epidemiological characteristics of recurrent HSP needing hospitalization. Recurrent cases of HSP
may be more common than reported, but most are treated in
an outpatient setting. In our inpatient population, no clinical
or laboratory characteristics were found to be predictive of
recurrence; the second episode was longer than the first one;
and the lag period between the 2 episodes was substantially
longer than previously reported.
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