Erythema multiforme associated with autoreactivity to 17 alpha-hydroxyprogesterone.
We report a case of a 23-year-old woman who was afflicted with disseminated skin erythema multiforme-like eruptions that started at the menarche, relapsed at the premenstrual periods, dramatically spread during two pregnancies and cleared after abortion; the skin lesions responded dramatically to thalidomide treatment. A high-affinity binding factor to 17 alpha-hydroxyprogesterone (17-OHP) was found in the serum of this patient. Her lymphocytes did not proliferate in vitro after exposure to exogenous 17-OHP but showed significant chromatin activation. There was a decreased expression of HLA antigens at the surface of the patient's blood lymphocytes. This is a unique well-documented case of erythema multiforme most possibly due to autoreactivity to 17-OHP; the precise mechanism(s) of this autoreactivity has not been established.
Pinto
J S
JS
Department of Dermatology, Faculty of Medical Sciences, Portuguese Cancer Institute, Lisbon.
Sobrinho
L
L
da Silva
M B
MB
Porto
M T
MT
Santos
M A
MA
Baló-Banga
M
M
Arala-Chaves
M
M
D019326:17-alpha-Hydroxyprogesterone; D000328:Adult; D004892:Erythema Multiforme / Q000188:drug therapy / Q000276:immunology*; D005260:Female; D006680:HLA Antigens / Q000032:analysis; D006801:Humans; D006908:Hydroxyprogesterones / Q000276:immunology*; D008214:Lymphocytes / Q000276:immunology; D008597:Menstrual Cycle; D013792:Thalidomide / Q000008:administration & dosage / Q000627:therapeutic use
D006680:HLA Antigens; D006908:Hydroxyprogesterones; D013792:Thalidomide; D019326:17-alpha-Hydroxyprogesterone