Acute febrile neutrophilic dermatosis

Definition:

Acute febrile neutrophilic dermatosis (AFND), also referred to as pyrin-associated autoinflammatory disease (PAAND), is an autosomal dominant autoinflammatory disorder characterized by fever and arthritis. Pyrin encoded by MEFV gene is a protein involved as a suppressor of inflammasome and mutations in the MEFV gene have been linked to AFND.

References:

[1]. Junya Masumoto, et al. Molecular biology of autoinflammatory diseases. Inflamm Regen. 2021 Oct 11;41(1):33. [Content Brief]

[2]. Mizuna Otsuka, et al. A case of neutrophilic dermatosis with MEFV gene variant and abnormal activation of peripheral blood monocytes: a case report. Immunol Med. 2019 Mar;42(1):45-49. [Content Brief]

[3]. Peter A Nigrovic, et al. Monogenic autoinflammatory disorders: Conceptual overview, phenotype, and clinical approach. J Allergy Clin Immunol. 2020 Nov;146(5):925-937. [Content Brief]

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