Nsaids Linked to IgA-Mediated Hypersensitivity Vasculitis and Purpura Fulminans-Like Eruption

Guy Levenberg; Jonathan Bleier; Avshalom Leibowitz; Ophira Salomon; Mudi Misgav; Nancy Agmon-Levin; Ronen Shavit

doi:10.12890/2023_004072

Nsaids Linked to IgA-Mediated Hypersensitivity Vasculitis and Purpura Fulminans-Like Eruption

Eur J Case Rep Intern Med. 2023 Oct 18;10(11):004072. doi: 10.12890/2023_004072. eCollection 2023.

Authors

Guy Levenberg¹, Jonathan Bleier², Avshalom Leibowitz^{1

2}, Ophira Salomon^{1

3}, Mudi Misgav^{1

3}, Nancy Agmon-Levin^{1

4}, Ronen Shavit^{1

4}

Affiliations

¹ Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
² Department of Internal Medicine D, Sheba Medical Center, Tel-Hashomer, Ramat Gan, Israel.
³ Thrombosis and Haemostasis Institute, Sheba Medical Center, Tel-Hashomer, Ramat Gan, Israel.
⁴ Clinical Immunology, Angioedema and Allergy Institute, Sheba Medical Center, Tel-Hashomer, Ramat Gan, Israel.

Abstract

Background: IgA vasculitis and hypersensitivity reactions following exposure to non-steroidal anti-inflammatory drugs (NSAIDs) are very rarely associated with purpura fulminans (PF). The latter is a coagulation event characterised by decreased levels of protein C and a rapidly progressive purpuric rash, often leading to ischaemia, amputations and death.

Case summary: A previously healthy 66-year-old man presented with a vasculitic rash and abdominal pain following exposure to naproxen (NSAID), which quickly deteriorated to purpura fulminans-like eruption and skin necrosis, mainly involving the face and hands. The presence of IgA sediments on skin biopsy and decreased levels of complement as well as protein C pointed to an immune-mediated inflammatory process. Dramatic clinical escalation with immediate risk to organs and life required an aggressive and broad-spectrum therapeutic approach in an intensive care setting. Clinical improvement and complete reconstitution of protein C were achieved following plasma exchange with fresh frozen plasma (FFP) and immunosuppression with glucocorticoids with no persistent organ damage.

Conclusions: This rare case illustrates the catastrophic cross links between NSAIDs, IgA-mediated hypersensitivity vasculitis and purpura fulminans-like syndrome. A high index of suspicion is required for the evaluation of environmental exposures such as drugs and infections in patients with vasculitis and/or purpura. A rapid and comprehensive therapeutic approach should be implemented to avoid multi-organ damage, amputations and death. Complete avoidance of the offending agent is key for future prevention of recurrence.

Learning points: This case illustrates a rare cross link between a commonly used drug (NSAIDs) and severe, life-threatening hypersensitivity reactions (IgA vasculitis and purpura fulminans-like eruption).These events require a high index of suspicion and emphasise the importance of considering environmental exposures such as drugs in the immediate diagnosis of both conditions.In addition to long-term drug avoidance, early and aggressive interventions are required to avoid organ damage, amputations or death.

Keywords: IgA vasculitis; NSAIDs; drug hypersensitivity; purpura fulminans; vasculitis.