[Hyperimmunoglobulinemia D or periodic fever syndrome. Cutaneous manifestations in 3 cases]

Abstract

Introduction: We describe 3 cases of hyperimmunoglobulinemia D syndrome (hyper-IgD syndrome).

Cases report: As the 2 first cases are conventional with an onset in childhood, the third one appeared later suggesting possibility of acquired syndrome. The 3 cases presented with one or several skin eruptions during febrile attacks.

Comments: Up to now, 64 cases of this syndrome have been described. Skin lesions are observed in 80 p. 100 of patients. In this particular clinical context, they must lead to the diagnosis. This 3 cases and the review of literature show that skin manifestations are polymorphic, transient, not very symptomatic and not correlated to IgD rates. Histological examination may reveal urticarial reaction with leucocytoclasic vasculitis, where IgD could directly or not interact.