Table 2.

Classification of monogenic autoinflammatory diseases based on the main cutaneous manifestation.

1	Maculopapular rashes or inflammatory plaques	Familial Mediterranean Fever (FMF)
		TNF receptor-associated periodic syndrome (TRAPS) Hyper-IgD syndrome/Mevalonate kinase deficiency (HIDS/MKD) Otulipenia/OTULIN-related autoinflammatory syndrome (ORAS) HOIL-1 deficiency
2	Urticarial rashes	Cryopyrin-associated periodic syndromes (CAPS) NLRP12-associated autoinflammatory disease (NLRP12-AD) PLCγ2-associated antibody deficiency and immune dysregulation (PLAID) NLRC4-associated autoinflammatory diseases (NRLC4-AD) Vibratory Urticaria
3	Pustular, pyogenic or neutrophilic dermatosis-like rashes	Pyogenic sterile arthritis, pyoderma gangrenosum and acne (PAPA) Syndromic forms of pyoderma gangrenosum Deficiency of IL-1 receptor antagonist (DIRA) Deficiency of IL-36 receptor antagonist (DITRA) CARD-14 mediated psoriasis (CAMPS) Majeed syndrome Pyrin-associated autoinflammation with neutrophilic dermatosis (PAAND) Singleton-Merten syndrome (SMS) ADAM17 deficiency AP1S3 and autoinflammatory psoriasis NFKB1-associated sterile familial autoinflammatory necrotizing fasciitis (FANF)
4	Panniculitis or subcutaneous nodules	Blau syndrome / Early-onset sarcoidosis Chronic atypical neutrophilic dermatitis with lipodystrophy and elevated temperature (CANDLE)
5	Vasculitis or vasculopathy	Deficiency of adenosine deaminase 2 (DADA2) STING-associated vasculopathy with onset in infancy (SAVI) Familial chilblain lupus Aicardi-Goutières syndrome (AGS) 1-7 Spodyloenchondrodysplasia with immune dysregulation (SPENCDI)
6	Hyperkeratotic lesions	NLRP-1 associated disease (NAIAD)
7	Hyperpigmented lesions	H syndrome
8	Bullous lesions	Autoinflammation and PLCγ2-associated antibody deficiency and immune dysregulation (APLAID)
9	Aphthous lesions	Haploinsufficiency of A20 (HA20) Autoinflammatory periodic fever, immunodeficiency and thrombocytopenia (PFIT) C/EBPε-associated autoinflammation and immune impairment of neutrophils (CAIN) NFKB1-associated Behcet-like disease RELA haploinsufficiency Monogenic forms of inflammatory bowel disease (IL-10 signaling defects)