A syndrome characterized by nodular eosinophilic infiltration of the skin and immunoglobulin isotype imbalance.
A 9-year-old boy had recurrent acute, plaquelike, nodular infiltrations with overlying vesicles and bullae of the face and right hand. Histologically, the lesions consisted of a perivascular and periadnexal lymphohistiocytic infiltrate with many eosinophils. The lesions responded to dapsone therapy. Laboratory studies revealed blood eosinophilia, hyperimmunoglobulinemias E and G4, and hypoimmunoglobulinemias M and G1-3, which normalized after treatment. The patient's immune deviation is consistent with a transient imbalance of lymphokine production in helper T cells.
Hauser
C
C
Department of Dermatology, Hôpital Cantonal Universitaire, Geneva, Switzerland.
Saurat
J H
JH
D002648:Child; D004406:Dysgammaglobulinemia / Q000150:complications*; D004802:Eosinophilia / Q000150:complications*; D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications*; D017099:IgG Deficiency; D007073:Immunoglobulin E; D007075:Immunoglobulin M / Q000172:deficiency; D008297:Male; D012867:Skin / Q000473:pathology; D012871:Skin Diseases / Q000150:complications / Q000276:immunology / Q000473:pathology*; D013577:Syndrome
D007075:Immunoglobulin M; D007073:Immunoglobulin E