Autoimmune Progesterone Dermatitis: A Case Report and Review of the Literature

Abstract

Autoimmune progesteron dermatitis (AIPD) is a rare disorder which is characterized by cyclical premenstrual flares of cutaneous or mucocutaneous manifestations. We present a 36-year-old woman with a 2-year history of oral ulcers and a rash which presents a week before every menstruation. She also had a history of recurrent herpes labialis infection; however herpes simplex virus could not be detected by PCR in oral lesions. Routine laboratory investigations, as well as serum complement levels were normal, except for elevated ANA titer (1/160). Histopathological examination of one of the papules was consistent with erythema multiforme and immunobullous diseases were ruled out by negative immunofluorescence studies. Based on the history and the clinical features, AIPD was suspected and the diagnosis was confirmed by flare of the lesions after progesterone challenge test. Subsequently, oral tamoxifen was started which controlled her flares significantly and no major side effects except amenorrhea was observed during treatment. Next she was given spironolactone which did not control her symptoms, therefore oral prednisolone had to be introduced. Due to its rare occurence, clinical features, pathogenesis, diagnostic and treatment alternatives of AIPD are discussed in this study based on the review of the medical literature. (Turkderm 2009, 43: 122-5)