Acquired C1 inhibitor deficiency revealing systemic lupus erythematosus.
We report the case of a previously healthy 54-year-old woman, without any family history, who developed an angioneurotic edema with acquired C1 inhibitor deficiency and systemic lupus erythematosus. The search for a lymphoproliferative disorder was negative. Hydroxychloroquine therapy induced simultaneous resolution of lupus and angioedema. The pathophysiology of this association is discussed.
Ochonisky
S
S
Service de Dermatologie, Hôpital Henri-Mondor, Créteil, France.
Intrator
L
L
Wechsler
J
J
Revuz
J
J
Bagot
M
M
D000799:Angioedema / Q000150:complications* / Q000188:drug therapy / Q000503:physiopathology; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency*; D005260:Female; D006801:Humans; D006886:Hydroxychloroquine / Q000627:therapeutic use; D007153:Immunologic Deficiency Syndromes / Q000188:drug therapy / Q000503:physiopathology; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000188:drug therapy / Q000503:physiopathology; D008875:Middle Aged
D003174:Complement C1 Inactivator Proteins; D006886:Hydroxychloroquine