[Hyperimmunoglobulinemia D or periodic fever syndrome. Cutaneous manifestations in 3 cases].
BACKGROUND
We describe 3 cases of hyperimmunoglobulinemia D syndrome (hyper-IgD syndrome).


METHODS
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.


CONCLUSIONS
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.
Cartier
H
H
Service de Dermatologie, CHRU, Brest.
Guillet
M H
MH
Lemoigne
E
E
Sassolas
B
B
Leroy
J P
JP
Mottier
D
D
Guillet
G
G
D000328:Adult; D018771:Arthralgia / Q000209:etiology; D010505:Familial Mediterranean Fever / Q000150:complications*; D005260:Female; D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications*; D007072:Immunoglobulin D; D008297:Male; D012871:Skin Diseases / Q000209:etiology* / Q000473:pathology
D007072:Immunoglobulin D
Syndrome hyper-IgD ou pseudo-maladie périodique. Manifestations cutanées dans 3 observations.