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

Autor:	Levenberg G; Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel., Bleier J; Department of Internal Medicine D, Sheba Medical Center, Tel-Hashomer, Ramat Gan, Israel., Leibowitz A; Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.; Department of Internal Medicine D, Sheba Medical Center, Tel-Hashomer, Ramat Gan, Israel., Salomon O; Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.; Thrombosis and Haemostasis Institute, Sheba Medical Center, Tel-Hashomer, Ramat Gan, Israel., Misgav M; Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.; Thrombosis and Haemostasis Institute, Sheba Medical Center, Tel-Hashomer, Ramat Gan, Israel., Agmon-Levin N; Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.; Clinical Immunology, Angioedema and Allergy Institute, Sheba Medical Center, Tel-Hashomer, Ramat Gan, Israel., Shavit R; Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.; Clinical Immunology, Angioedema and Allergy Institute, Sheba Medical Center, Tel-Hashomer, Ramat Gan, Israel.
Jazyk:	angličtina
Zdroj:	European journal of case reports in internal medicine [Eur J Case Rep Intern Med] 2023 Oct 18; Vol. 10 (11), pp. 004072. Date of Electronic Publication: 2023 Oct 18 (Print Publication: 2023).
DOI:	10.12890/2023_004072
Abstrakt:	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. Competing Interests: Conflicts of Interests: The Authors declare that there are no competing interests. (© EFIM 2023.)
Databáze:	MEDLINE
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