Cryopyrin-associated periodic syndrome (CAPS) is a monogenetic auto-inflammatory disorder with systemic manifestations. Innate immune system aberrance is seen in both CAPS and erythema nodosum and this case may point to a previously unknown association. In pediatric patients with EN and systemic features, the possibility of underlying CAPS should be considered.
Keywords: Muckle‐Wells syndrome; auto‐inflammation; chronic infantile neurological; cryopoyrin‐associated periodic syndrome; cutaneous and articular syndrome; erythema nodosum.