Bullous Henoch-Schönlein Purpura: A Case Series

Giulia Paloni, Valeria Silecchia, Irene Berti, Arianna Tagarelli, Elisa Mazzoni, Andrea Lambertini, Enrico Valerio, Ramon Grimalt, Mario Cutrone


Henoch-Schönlein purpura (HSP) represents the most common vasculitis in children. Typically, HSP manifests with palpable purpura and edema, usually following an urticarial or erythematous maculopapular eruption. Bullous HSP represents an uncommon presentation of “classic” HSP, exhibited by only 2% of affected children. We here present three cases of bullous HSP. This variant seems due to an excess of production of neutrophilic matrix metalloproteinase-9, a lytic enzyme that degrades basement membrane components, eventually leading to dermoepidermal detachment and blister formation. Notably, American College of Rheumatology (ACR), Paediatric Rheumatology European Society (PRES), European League against Rheumatism (EULAR), and Paediatric Rheumatology INternational Trials Organization (PRINTO) do not encompass blisters and/or bullae into HSP classification criteria. Outcome of bullous HSP does not differ from “classic” purpuric HSP. The former, however, demands special care measures: local pain control, topic antibiotic therapy, and protective dressings over areas of open or blistered skin.

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