Caso atipico di orticaria
1U.O. Medicina di Laboratorio, Azienda Ospedaliera - Università degli Studi di Padova
2U.O. Ematologia e Immunologia Clinica, Azienda Ospedaliera di Padova
3Dipartimento di Medicina, Università degli Studi di Padova
A case of atypical urticaria
Adult onset Still’s disease (AOSD) is a rare clinical entity with unknown etiology and pathogenesis, characterized by high spiking fevers, arthritis, typical evanescent, nonpruritic, macular and salmon
coloured rash, and multiorgan involvement. AOSD remains a diagnostic challenge due to the overlapping features with numerous infective, neoplastic and rheumatological conditions. A case of AOSD with atypical cutaneous manifestations in a 50-year-old female is reported. The patient’s symptoms included: remittent fever, widespread urticarial, maculopapular erythema and a sore throat. Hematological investigations showed leukocytosis with neutrophilia. There were markedly elevated levels of serum ferritin (20.920 μg/L) and mild liver dysfunction. A glycosylated ferritin assay was performed because of a severe hyperferritinemia, showing a reduction of the glycosylated fraction (<20%), a newly proposed diagnostic criteria for AOSD. We emphasize the diagnostic value of low glycosylated ferritin concentration for the differential diagnosis of this rare disease, particularly in case of atypical presentation.