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JCR: Journal of Clinical Rheumatology:
doi: 10.1097/01.rhu.0000255692.46165.19
Original Articles

Recurrent Henoch-Schönlein Purpura in Children

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

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From the Department of Pediatrics C, Schneider Children's 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 Children's Medical Center of Israel, 49202 Petah Tiqva, Israel. E-mail: dariop@clalit.org.il

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Abstract

Background: Henoch-Schönlein 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 (2.2–10 years) at the second one, with a mean lag period of 13.5 ± 2.8 months (range 2–26). 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.

Henoch-Schönlein 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 (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 immune-complex-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.

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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 Rheumatology's 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 statistical significance with the Fisher exact test and Student t test.

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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 1–14.8 years). Mean duration of the skin involvement was 11.5 days (range 3–27 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.

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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.

Table 1
Table 1
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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 2.2–10 years). Mean lag period between the 2 episodes was 13.5 ± 2.8 months (range 2–26 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.

On long-term follow-up (at least 2 years), no further recurrent episodes were reported, and no complications or persistent symptoms were found.

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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 (Schönlein). 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 16-year-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 2–3 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 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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REFERENCES

1. Saulsbury FT. Henoch-Schönlein purpura in children. Report of 100 patients and review of the literature. Medicine. 1999;78:395–409.

2. Nussinovitch M, Prais D, Finkelstein Y, et al. Cutaneous manifestations of Henoch-Schönlein purpura in young children. Pediat Dermatol. 1998;5:426–428.

3. Kaufman RH, Hermann WA, Meyer CJL, et al. Circulating IgA immune complexes in Henoch-Schönlein purpura. Am J Med. 1980;69:859–866.

4. Nathwani D, Laing R, Smith CC, et al. Recurrent post-infective Henoch-Schönlein syndrome: a genetic influence related to HLA B35? J Infect. 1992;25:205–210.

5. Michel BA, Hunder GG, Bloch DA, et al. Hypersensitivity vasculitis and Henoch-Schönlein purpura: a comparison between the 2 disorders. J Rheumatol. 1992;19:721–728.

6. Duzova A, Toaloglu R, Sanal O, et al. Henoch-Schönlein purpura in Wiskott-Aldrich syndrome. Pediatr Nephrol. 2001;16:5000–5002.

7. Nathan K, Cunasekaran TS, Berman JH. Recurrent gastrointestinal Henoch-Schoenlein purpura. J Clin Gastroenterol. 1999;29:86–89.

8. Pomeranz A, Korzets Z, Eliakim R, et al. Relapsing Henoch-Schönlein purpura associated with a tubo-ovarian abscess due to Morganella morganii. Am J Nephrol. 1997;17:471–473.

9. Hardoff D, Jaffe M, Front H. Recurrent episodes of testicular swelling preceding Henoch-Schönlein purpura by 11 months. Eur J Pediatr. 1987;146:613–614.

10. 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 Rhem. 2005;35:143–153.

Figure. No caption available.


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Keywords:

vasculitis; anaphylactoid; recurrence; prognosis; Henoch-Schonlein

© 2007 Lippincott Williams & Wilkins, Inc.

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