Henoch-Schönlein purpura and systemic disease in children: Retrospective study of clinical findings, histopathology and direct immunofluorescence in 34 paediatric patients

E. F. Johnson, Julia Lehman, D. A. Wetter, C. M. Lohse, Megha M Tollefson

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Abstract

Background Henoch-Schönlein purpura (HSP), an IgA-mediated small vessel vasculitis, is the most common form of vasculitis in children. HSP is commonly associated with systemic involvement of the gastrointestinal tract, joints and kidneys. Renal involvement is the main cause of morbidity and mortality in HSP. Objectives To characterize the clinical, histopathological and direct immunofluorescence (DIF) findings, and to correlate the findings with systemic disease in 34 children with HSP seen at our institution. Methods This was a retrospective review of paediatric patients with HSP and with available biopsy specimens seen at our institution between 1993 and 2013. Results Thirty-four paediatric patients were identified (mean age 10·7 years). Renal involvement was found in 17 (50%) patients, gastrointestinal tract involvement in 22 (65%) and joint involvement in 23 (68%). Renal involvement was significantly associated with papillary dermal oedema on histopathology (P < 0·01) and the presence of perivascular C3 on DIF (P = 0·01). The presence of lesions above the waist was significantly associated with gastrointestinal involvement (P = 0·03), as was the presence of clinically apparent oedema (P = 0·01). Conclusions This study suggests that in children with HSP, microscopic dermal oedema and C3 on DIF may be predictive of renal involvement. Patients with clinically apparent oedema and lesions above the waist are more likely to have gastrointestinal involvement. What's already known about this topic? Henoch-Schönlein purpura (HSP) is commonly associated with systemic involvement. What does this study add? Lesions above the waist and clinically evident oedema are associated with gastrointestinal involvement. Renal involvement is associated with perivascular C3 on direct immunofluorescence and with microscopic dermal oedema. Clinical and microscopic findings may help predict systemic involvement in HSP in children.

Original languageEnglish (US)
Pages (from-to)1358-1363
Number of pages6
JournalBritish Journal of Dermatology
Volume172
Issue number5
DOIs
StatePublished - May 1 2015

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Schoenlein-Henoch Purpura
Direct Fluorescent Antibody Technique
Retrospective Studies
Pediatrics
Edema
Kidney
Vasculitis
Skin
Gastrointestinal Tract
Joints
Immunoglobulin A
Morbidity
Biopsy
Mortality

ASJC Scopus subject areas

  • Dermatology
  • Medicine(all)

Cite this

@article{8020ba41c67b4596bc8049976f8711fe,
title = "Henoch-Sch{\"o}nlein purpura and systemic disease in children: Retrospective study of clinical findings, histopathology and direct immunofluorescence in 34 paediatric patients",
abstract = "Background Henoch-Sch{\"o}nlein purpura (HSP), an IgA-mediated small vessel vasculitis, is the most common form of vasculitis in children. HSP is commonly associated with systemic involvement of the gastrointestinal tract, joints and kidneys. Renal involvement is the main cause of morbidity and mortality in HSP. Objectives To characterize the clinical, histopathological and direct immunofluorescence (DIF) findings, and to correlate the findings with systemic disease in 34 children with HSP seen at our institution. Methods This was a retrospective review of paediatric patients with HSP and with available biopsy specimens seen at our institution between 1993 and 2013. Results Thirty-four paediatric patients were identified (mean age 10·7 years). Renal involvement was found in 17 (50{\%}) patients, gastrointestinal tract involvement in 22 (65{\%}) and joint involvement in 23 (68{\%}). Renal involvement was significantly associated with papillary dermal oedema on histopathology (P < 0·01) and the presence of perivascular C3 on DIF (P = 0·01). The presence of lesions above the waist was significantly associated with gastrointestinal involvement (P = 0·03), as was the presence of clinically apparent oedema (P = 0·01). Conclusions This study suggests that in children with HSP, microscopic dermal oedema and C3 on DIF may be predictive of renal involvement. Patients with clinically apparent oedema and lesions above the waist are more likely to have gastrointestinal involvement. What's already known about this topic? Henoch-Sch{\"o}nlein purpura (HSP) is commonly associated with systemic involvement. What does this study add? Lesions above the waist and clinically evident oedema are associated with gastrointestinal involvement. Renal involvement is associated with perivascular C3 on direct immunofluorescence and with microscopic dermal oedema. Clinical and microscopic findings may help predict systemic involvement in HSP in children.",
author = "Johnson, {E. F.} and Julia Lehman and Wetter, {D. A.} and Lohse, {C. M.} and Tollefson, {Megha M}",
year = "2015",
month = "5",
day = "1",
doi = "10.1111/bjd.13472",
language = "English (US)",
volume = "172",
pages = "1358--1363",
journal = "British Journal of Dermatology",
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}

TY - JOUR

T1 - Henoch-Schönlein purpura and systemic disease in children

T2 - Retrospective study of clinical findings, histopathology and direct immunofluorescence in 34 paediatric patients

AU - Johnson, E. F.

AU - Lehman, Julia

AU - Wetter, D. A.

AU - Lohse, C. M.

AU - Tollefson, Megha M

PY - 2015/5/1

Y1 - 2015/5/1

N2 - Background Henoch-Schönlein purpura (HSP), an IgA-mediated small vessel vasculitis, is the most common form of vasculitis in children. HSP is commonly associated with systemic involvement of the gastrointestinal tract, joints and kidneys. Renal involvement is the main cause of morbidity and mortality in HSP. Objectives To characterize the clinical, histopathological and direct immunofluorescence (DIF) findings, and to correlate the findings with systemic disease in 34 children with HSP seen at our institution. Methods This was a retrospective review of paediatric patients with HSP and with available biopsy specimens seen at our institution between 1993 and 2013. Results Thirty-four paediatric patients were identified (mean age 10·7 years). Renal involvement was found in 17 (50%) patients, gastrointestinal tract involvement in 22 (65%) and joint involvement in 23 (68%). Renal involvement was significantly associated with papillary dermal oedema on histopathology (P < 0·01) and the presence of perivascular C3 on DIF (P = 0·01). The presence of lesions above the waist was significantly associated with gastrointestinal involvement (P = 0·03), as was the presence of clinically apparent oedema (P = 0·01). Conclusions This study suggests that in children with HSP, microscopic dermal oedema and C3 on DIF may be predictive of renal involvement. Patients with clinically apparent oedema and lesions above the waist are more likely to have gastrointestinal involvement. What's already known about this topic? Henoch-Schönlein purpura (HSP) is commonly associated with systemic involvement. What does this study add? Lesions above the waist and clinically evident oedema are associated with gastrointestinal involvement. Renal involvement is associated with perivascular C3 on direct immunofluorescence and with microscopic dermal oedema. Clinical and microscopic findings may help predict systemic involvement in HSP in children.

AB - Background Henoch-Schönlein purpura (HSP), an IgA-mediated small vessel vasculitis, is the most common form of vasculitis in children. HSP is commonly associated with systemic involvement of the gastrointestinal tract, joints and kidneys. Renal involvement is the main cause of morbidity and mortality in HSP. Objectives To characterize the clinical, histopathological and direct immunofluorescence (DIF) findings, and to correlate the findings with systemic disease in 34 children with HSP seen at our institution. Methods This was a retrospective review of paediatric patients with HSP and with available biopsy specimens seen at our institution between 1993 and 2013. Results Thirty-four paediatric patients were identified (mean age 10·7 years). Renal involvement was found in 17 (50%) patients, gastrointestinal tract involvement in 22 (65%) and joint involvement in 23 (68%). Renal involvement was significantly associated with papillary dermal oedema on histopathology (P < 0·01) and the presence of perivascular C3 on DIF (P = 0·01). The presence of lesions above the waist was significantly associated with gastrointestinal involvement (P = 0·03), as was the presence of clinically apparent oedema (P = 0·01). Conclusions This study suggests that in children with HSP, microscopic dermal oedema and C3 on DIF may be predictive of renal involvement. Patients with clinically apparent oedema and lesions above the waist are more likely to have gastrointestinal involvement. What's already known about this topic? Henoch-Schönlein purpura (HSP) is commonly associated with systemic involvement. What does this study add? Lesions above the waist and clinically evident oedema are associated with gastrointestinal involvement. Renal involvement is associated with perivascular C3 on direct immunofluorescence and with microscopic dermal oedema. Clinical and microscopic findings may help predict systemic involvement in HSP in children.

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U2 - 10.1111/bjd.13472

DO - 10.1111/bjd.13472

M3 - Article

C2 - 25308217

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VL - 172

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EP - 1363

JO - British Journal of Dermatology

JF - British Journal of Dermatology

SN - 0007-0963

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