. 2023 Jan 3;7:8–16. doi: 10.5414/ALX02375E

Table 3. Further selected autoinflammatory diseases that may be associated with wheals.

Monogenetic autoinflammatory diseases	Complex autoinflammatory diseases
With wheals – Familial Mediterranean fever (FMF)* – Hyper-IgD syndrome/mevalonate kinase deficiency (HIDS/MKD)* – Tumor necrosis factor (TNF) receptor-associated periodic syndrome (TRAPS)* – VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome* With cold-induced wheals – NLRP12-associated cold-induced autoinflammatory syndrome (FCAS 2) – NLRC4-associated cold-induced autoinflammatory syndrome (FCAS 3) – PLCG2-associated antibody deficiency and immune dysregulation (PLAID) – Factor XII-associated cold-induced autoinflammatory syndrome (FACAS)	– Systemic juvenile idiopathic arthritis (SJIA)/adult onset Still’s disease (AOSD)* – Sweet syndrome*

Monogenetic autoinflammatory diseases

Complex autoinflammatory diseases

With wheals
– Familial Mediterranean fever (FMF)*
– Hyper-IgD syndrome/mevalonate kinase deficiency (HIDS/MKD)*
– Tumor necrosis factor (TNF) receptor-associated periodic syndrome (TRAPS)*
– VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome*

With cold-induced wheals
– NLRP12-associated cold-induced autoinflammatory syndrome (FCAS 2)
– NLRC4-associated cold-induced autoinflammatory syndrome (FCAS 3)
– PLCG2-associated antibody deficiency and immune dysregulation (PLAID)
– Factor XII-associated cold-induced autoinflammatory syndrome (FACAS)

– Systemic juvenile idiopathic arthritis (SJIA)/adult onset Still’s disease (AOSD)*
– Sweet syndrome*

*The appearance of wheals is not obligatory for these diseases.