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.
Category
Immune system disease
Brite
Human diseases in ICD-11 classification [BR:br08403]
14 Diseases of the skin
Inflammatory dermatoses
Inflammatory erythemas and other reactive inflammatory dermatoses
Neutrophilic dermatoses
EB20 Acute febrile neutrophilic dermatosis
H02590 Acute febrile neutrophilic dermatosis
Pathway-based classification of diseases [BR:br08402]
Immune system
nt06521 NLR signaling
H02590 Acute febrile neutrophilic dermatosis
Otsuka M, Koga T, Sumiyoshi R, Koike Y, Furukawa K, Okamoto M, Endo Y, Tsuji S, Takatani A, Shimizu T, Igawa T, Kawashiri SY, Iwamoto N, Ichinose K, Tamai M, Nakamura H, Origuchi T, Daisuke N, Kawakami A
Title
A case of neutrophilic dermatosis with MEFV gene variant and abnormal activation of peripheral blood monocytes: a case report.
