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Cutaneous Manifestations of Small-Vessel Leukocytoclastic Vasculitides in Childhood

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Abstract

In childhood, cutaneous small-vessel vasculitides include Henoch–Schönlein syndrome, a systemic vasculitis, and Finkelstein–Seidlmayer syndrome, a skin-limited vasculitis. Both Henoch–Schönlein and Finkelstein–Seidlmayer syndromes are seen more frequently in white or Asian compared with black children and occur especially in winter and spring with a male-to-female ratio of approximately 2:1. In everyday clinical practice, both conditions are diagnosed on clinical grounds without histological confirmation. The characteristic cutaneous hallmarks of Henoch–Schönlein syndrome include a purpuric rash in all and a subcutaneous edema in approximately every second case, which are often preceded by non-specific red or pink macular elements that mimic a non-itching urticarial rash. Recent data point out that Henoch–Schönlein children often present further cutaneous findings such as Köbnerization, Rumpel–Leede capillary fragility phenomenon, and blistering eruptions. Children with Finkelstein–Seidlmayer syndrome are usually ≤24 months of age and not ill-appearing. They present with (a) large, round, red to purpuric plaques (often with a targetoid appearance) predominantly over the cheeks, ears, and extremities and (b) often tender non-pitting edema of the distal extremities, ears, and face (without pruritus). Both in Henoch–Schönlein syndrome and Finkelstein–Seidlmayer syndrome, there is often scrotal involvement. The cutaneous findings remit without sequelae within 2 months in Henoch–Schönlein and 3 weeks in Finkelstein–Seidlmayer syndrome.

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Notes

  1. This condition received the name Wegner granulomatosis in the 1930s, when the German pathologist Friedrich Wegener (1907–1990) published a comprehensive description of the condition. It was recently decided to abandon the term Wegener’s granulomatosis on the evidence that Wegener was an active member of the Nazi party before and during World War II.

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Acknowledgments

The authors thank the patients and their families for permitting to publish the images.

The authors would like to thank Dr. Alec Villa for his assistance in the linguistic revision.

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Author notes

  1. Sebastiano A. G. Lava and Gregorio P. Milani contributed equally to this work.

Authors and Affiliations

  1. Department of Pediatrics, University Children’s Hospital of Bern, Inselspital, Bern, Switzerland

    Sebastiano A. G. Lava

  2. Pediatric Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico and Department of Clinical Sciences and Community Health, Università degli Studi di Milano, Milan, Italy

    Gregorio P. Milani

  3. Pediatric Emergency Department, Università degli Studi di Milano, Foundation IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, Milan, Italy

    Emilio F. Fossali

  4. Pediatric Department of Southern Switzerland, San Giovanni Hospital, 6500, Bellinzona, Switzerland

    Giacomo D. Simonetti & Mario G. Bianchetti

  5. Università della Svizzera Italiana, Lugano, Switzerland

    Giacomo D. Simonetti & Mario G. Bianchetti

  6. Pediatric Intermediate Care Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico and Department of Clinical Sciences and Community Health, Università degli Studi di Milano, Milan, Italy

    Carlo Agostoni

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Correspondence to Mario G. Bianchetti.

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The present article does not contain any studies with human participants or animals performed by any of the authors.

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This work was supported in part by the Fondazione E. Balli and the Swiss Research Network of Clinical Pediatric Hubs

Dr. Gregorio P. Milani is the current recipient of research grants from the Fondazione G. & D. de Marchi ONLUS.

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Lava, S.A.G., Milani, G.P., Fossali, E.F. et al. Cutaneous Manifestations of Small-Vessel Leukocytoclastic Vasculitides in Childhood. Clinic Rev Allerg Immunol 53, 439–451 (2017). https://doi.org/10.1007/s12016-017-8626-3

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