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Blistering eruptions in childhood Henoch-Schönlein syndrome: systematic review of the literature

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Abstract

The occurrence of blistering eruptions in childhood Henoch-Schönlein syndrome has been so far addressed exclusively in individual case reports. To describe epidemiology, clinical presentation, and therapeutic options in Henoch-Schönlein patients ≤18 years of age with blistering eruptions, we completed a systematic literature search. For the final analysis, we retained 39 reports. Ten children with blisters were found in 7 (1.5%) case series containing a total of 666 unselected pediatric Henoch-Schönlein cases. We also found 41 individually documented cases of Henoch-Schönlein syndrome with blistering eruptions. Blistering eruptions and purpura were distributed very similarly, blisters developed concomitantly with palpable purpura or with a latency of ≤14 days, and 80% of the cases remitted within 4 weeks with a similar course in children managed expectantly and in those managed with steroids.

Conclusion: Blistering eruptions are rare in Henoch-Schönlein syndrome. They can be a source of diagnostic dilemma but do not have any prognostic value since they almost always spontaneously subside within 4 weeks.

What is known:

• Textbooks and reviews marginally refer to the occurrence of blistering eruptions in children with Henoch-Schönlein syndrome.

What is new

• Blistering eruptions occur in <2% of cases.

• Blisters and purpura are distributed similarly, blisters develop concomitantly with purpura or with a latency of ≤14 days.

• Almost all cases remit within 4 weeks with a similar course in children managed expectantly and in those managed with systemic steroids.

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Acknowledgments

The study was supported by the Fondazione Ettore Balli. S.A.G.L. is the current recipient of research grants from the Fondazione Ettore e Valeria Rossi and the Swiss National Science Foundation. The authors would like to thank Dr. Alec Villa for his assistance in the linguistic revision.

Authors’ Contributions

– Study concept and design: V.R., S.A.G.L., M.G.B., G.P.R.

– Acquisition, analysis, and interpretation of data: V.R., S.A.G.L., G.D.S., M.G.B., G.P.M.

– Statistical analysis: G.D.S., M.G.B., G.P.M.

– Drafting of the manuscript: V.R., S.A.G.L., M.G.B., G.P.R.

– Critical revision of the manuscript: V.R., S.A.G.L., G.D.S., M.G.B., G.P.R., G.P.M.

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Authors and Affiliations

  1. Pediatric Department of Southern Switzerland, 6500, Bellinzona, Switzerland

    Vera Ramelli, Giacomo D. Simonetti, Mario G. Bianchetti & Gian Paolo Ramelli

  2. University Children’s Hospital Bern and University of Bern, Bern, Switzerland

    Sebastiano A. G. Lava & Giacomo D. Simonetti

  3. Pediatric Pharmacology and Pharmacogenetics, Hôpital Robert Debré, Paris, France

    Sebastiano A. G. Lava

  4. Università della Svizzera Italiana, Lugano, Switzerland

    Mario G. Bianchetti

  5. Pediatric unit, Università degli Studi di Milano, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy

    Gregorio P. Milani

Authors
  1. Vera Ramelli
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  2. Sebastiano A. G. Lava
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  4. Mario G. Bianchetti
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  5. Gian Paolo Ramelli
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  6. Gregorio P. Milani
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Corresponding author

Correspondence to Mario G. Bianchetti.

Ethics declarations

The study has been performed in accordance with the ethical standards as laid down in the 1964 Declaration of Helsinki and its later amendments.

Conflict of interest

The authors declare that they have no competing interests.

Additional information

Communicated by David Nadal

V.R. and S.A.G.L. contributed equally to this work.

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Ramelli, V., Lava, S.A.G., Simonetti, G.D. et al. Blistering eruptions in childhood Henoch-Schönlein syndrome: systematic review of the literature. Eur J Pediatr 176, 487–492 (2017). https://doi.org/10.1007/s00431-017-2858-3

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  • DOI: https://doi.org/10.1007/s00431-017-2858-3

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