Table 5.

Onset and general manifestations of NFKB-diseases (‘relopathies’) in childhood.

	Onset	Clinical signs observed in children at onset
Blau syndrome (BS)	First infancy	Brown-coloured scaly and ichthyosis-like or lichenoid rashes, recurrent polyarthritis, granulomatous uveitis (anterior, posterior or intermediate), risk of ocular sequelae (synechiae, cataracts, band keratopathy)
Deficiency of the interleukin-36 receptor antagonist (DITRA)	Variable (many cases may start in the first infancy)	Generalized severe pustular psoriasis, acute generalized exanthematous pustulosis, pustulosis of palms and soles, disseminated subcorneal pustules, Hallopeau’s acrodermatitis continua, recurrent fevers
CARD14-mediated psoriasis (CAMPS)	Variable (many cases may start in the first infancy)	Plaque psoriasis, pityriasis rubra pilaris, pustular psoriasis, joint pain, recurrent fevers
OTULIN-related autoinflammatory syndrome (ORAS)	First infancy	Erythematous skin rash with nodules, joint and abdominal pain, diarrhea, lymph node enlargement, stunted growth, recurrent fevers
Haploinsufficiency of A20 (HA20)	First or second decade	Early-onset Behçet’s-like disease signs as aphthous stomatitis, oral and genital ulcers and/or uveitis combined with diffuse lymphadenopathy, arthritis, recurrent fevers