Autoimmune Progesterone Dermatitis

Tami Maguire

Disclosures

Dermatology Nursing. 2009;21(4):190-192. 

In This Article

Abstract and Introduction

Abstract

Autoimmune progesterone dermatitis (APD) is a rare disorder characterized by recurrent skin manifestations starting during the luteal phase of a woman's menstrual cycle. Risk factors include fertile women with a previous history of exogenous progesterone intake and sometimes with pregnancy (Rodenas, Herranz, & Tercedor, 1998). Signs and symptoms of APD can present as urticaria, angioedema, erythema multiforme, and eczematous lesions, thought to be an unusual allergy to progesterone. Treatment options include a variety of medications used to induce anovulation and ultimately oophorectomy for intractable symptoms and difficult management.

Introduction

A patient presents to your office with very painful ulcers inside her mouth. A cluster of red, raised lesions on the lower aspect of the left scapula and target lesions on the palms of her hands are also present. She explains that a few months earlier she had been diagnosed with what was thought to be Stevens-Johnson syndrome and was started on prednisone, which relieved her symptoms. She reports her symptoms begin approximately 5 to 7 days prior to the start of her menses and resolve approximately 4 to 5 days after the end of her menses. The lesions are erythema multiforme and urticarial. The oral mucosal lesions are red, raw, and extend to the outer part of the lips. What is the critical thinking and differential diagnosis that should be considered?

According to the published literature, approximately 50 cases of autoimmune progesterone dermatitis (APD) have been documented. Because of the limited number of reported cases, the National Institutes of Health (NIH) has classified this disorder as a rare disease, meaning less than 200,000 cases have ever been reported. The NIH's Office of Rare Diseases indicated no active clinical trials or support groups for this condition. While many women complain of worsening acne and water retention during menstruation, there are a small number of women for whom the menstrual cycle is associated with a variety of skin manifestations such as urticaria, folliculitis, and angioedema (Baptist & Baldwin, 2004). It is reasonable to consider many cases of APD are undiagnosed due to the generic nature of the signs and symptoms and the association with menstruation. The purpose of this article is to educate dermatology nurse practitioners on the signs, symptoms, pathogenesis, diagnosis, and treatment of APD.

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