Erythema multiforme Associated with Autoreactivity to 17α-Hydroxyprogesterone

Abstract

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 17α-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.

The histopathologic features of autoimmune progesterone dermatitis

“…Autoimmunity is favored as many of these patients experience a flare with exposure to progesterone and cessation of symptoms with removal of progesterone . In addition, antibodies to progesterone have been showed in some patients …”

Thalidomide: dermatological indications, mechanisms of action and side-effects

“…After thalidomide administration, two patients with recurrent erythema multiforme had fewer and milder episodes 56 . A 23‐year‐old woman with disseminated skin erythema multiforme eruptions that had started at menarche and spread during two pregnancies responded well to thalidomide treatment 57 . Two other patients responded to thalidomide 100 mg daily, but later relapsed after tapering the drug and eventually discontinued therapy due to neuropathy 58 .…”

Chronic urticaria associated with autoreactivity to progesterone

“…In one the chroniatin activation test, that reflects lymphocyte reactivity [18]. was positive to l7-a:-hydroxyprogesterone and negative to progesterone [8].…”