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Autoimmune Progesterone Dermatitis Mistaken for Stevens-Johnson Syndrome

Drayer, Sara M. MD; Laufer, Larry R. MD; Farrell, Maureen E. MD

doi: 10.1097/AOG.0000000000002537
Contents: Case Reports

BACKGROUND: Autoimmune progesterone dermatitis is an uncommon disease presenting with cyclical skin eruptions corresponding with the menstrual cycle luteal phase. Because symptoms are precipitated by rising progesterone levels, treatment relies on hormone suppression.

CASE: A 22-year-old nulligravid woman presented with symptoms mistaken for Stevens-Johnson syndrome. A cyclic recurrence of symptoms occurred, and the diagnosis of autoimmune progesterone dermatitis was made by an intradermal progesterone challenge. After 48 months, her disease remained refractory to medical management, and definitive surgical treatment with bilateral oophorectomy was performed.

CONCLUSIONS: Autoimmune progesterone dermatitis is a challenging diagnosis as a result of its rarity and variety of clinical presentations. Treatment centers on suppression of endogenous progesterone and avoidance of exogenous triggers. When these modalities fail, surgical management must be undertaken.

When autoimmune progesterone dermatitis medical treatment fails, surgical management is an option.

Departments of Obstetrics and Gynecology and Reproductive Endocrinology and Infertility, Naval Medical Center San Diego, San Diego, California.

Corresponding author: Sara M. Drayer, MD, Naval Medical Center San Diego, 34800 Bob Wilson Drive, Building 3, Floor 1, San Diego, CA 92134; email:

Financial Disclosure The authors did not report any potential conflicts of interest.

This article replaces Drayer SM, Laufer LR, Farrell ME. Autoimmune progesterone dermatitis presenting as Stevens-Johnson syndrome. Obstet Gynecol 2017;130:881–4. For more information, see Dr. Drayer's reply to a Letter (p. 739) and the retraction and replacement notice (p. 746) in this issue.

The views expressed are those of the authors and do not necessarily reflect the official policy of the Department of the Navy, Department of Defense, or the U.S. Government.

Each author has indicated that he or she has met the journal's requirements for authorship.

© 2018 by American College of Obstetricians and Gynecologists. Published by Wolters Kluwer Health, Inc. All rights reserved.