Successful treatment with anakinra in generalized spiculated porokeratosis and severe hidradenitis suppurativa in a patient with <i>MVK</i> and <i>MEFV</i> mutations
Ayşe Öktem, Günel Rasulova, Büşra Çavdarlı, Seher Bostancı, Aylın Okçu Heper, Seçil Vural
Abstract
The synergistic effect of two mutations of the innate immune system may facilitate hyperactivation and dysregulation of the inflammasome in skin macrophages. This case connects two diseases, hidradenitis suppurativa and porokeratosis, by the central role of autoinflammation.
Topics & Concepts
Hidradenitis suppurativaMedicineDermatologyMEFVPityriasis rubra pilarisInflammasomePalmoplantar pustulosisAnakinraImmunologyMutationInflammationPsoriasisPathologyGene mutationDiseaseGeneticsBiologyGeneHidradenitis Suppurativa and TreatmentsAutoimmune and Inflammatory DisordersDermatological and Skeletal Disorders