title: A hyper-ferritinemia syndrome evolving in recurrent macrophage activation syndrome, as an onset of amyopathic juvenile dermatomyositis: a challenging clinical case in light of the current diagnostic criteria.
abstract: Juvenile dermatomyositis is an immune-mediated inflammatory multi-system disease involving mainly striated muscles and skin. Typical dermatological features are fundamental to establish the diagnosis, especially whenever the myopathy is very mild or absent, as it occurs in the form called as amyopathic juvenile dermatomyositis. Sometimes, systemic rheumatic diseases can develop a hyperferritinemia syndrome characterized by hemophagocytosis, namely macrophage activation syndrome, which represents a severe and life-threatening complication. Here, we describe a complex clinical history characterized by a hyper-ferritinemia syndrome after infectious mononucleosis, leading to recurrent episodes of macrophage activation syndrome. Finally, the late onset of several skin changes brought to a diagnosis of amyopathic juvenile dermatomyositis.
authors.affiliation: Department of Pediatrics, Azienda Ospedaliera di Melegnano, Milan, Italy. Electronic address: dimimedpv@yahoo.it.
authors.forename: Dimitri
authors.initials: D
authors.lastname: Poddighe
authors.affiliation: Department of Rheumatology, IRCCS Fondazione Policlinico San Matteo di Pavia, Italy.
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Cavagna
authors.affiliation: Department of Dermatology, IRCCS Fondazione Policlinico San Matteo di Pavia, Italy.
authors.forename: Valeria
authors.initials: V
authors.lastname: Brazzelli
authors.affiliation: Department of Pediatrics, Azienda Ospedaliera di Melegnano, Milan, Italy.
authors.forename: Paola
authors.initials: P
authors.lastname: Bruni
authors.affiliation: Department of Pediatrics, IRCCS Fondazione Policlinico San Matteo e Universita' degli Studi di Pavia, Italy.
authors.forename: Gian Luigi
authors.initials: GL
authors.lastname: Marseglia
mesh_terms: D000293:Adolescent; D017668:Age of Onset; D003882:Dermatomyositis / Q000175:diagnosis*; D006801:Humans; D055501:Macrophage Activation Syndrome / Q000175:diagnosis*; D012008:Recurrence; D012867:Skin / Q000473:pathology
keywords: Epstein–Barr virus; Hyperferritinemia; Juvenile dermatomyositis; Macrophage activation syndrome