A Case of Autoimmune progesterone dermatitis post-intrauterine device placement in rural occupied Palestine

Abstract

Autoimmune progesterone dermatitis is considered a rare cyclical autoimmune reaction to endogenous progesterone in fertile females that is characterized by skin lesions ranging from mild urticaria to anaphylaxis. A 30-year-old woman who had an intrauterine device placed, presented to her family medicine clinic with erythema and some edematous lesions, and pruritus. The skin biopsy showed perivascular infiltrate with neutrophils and was diagnosed as autoimmune progesterone dermatitis. Treatment included intrauterine device removal and treatment with tamoxifen. This is the first documented case of autoimmune progesterone dermatitis in occupied Palestine.

Introduction

Autoimmune progesterone dermatitis (APD) is considered a rare autoimmune reaction to progesterone in females that is characterized by skin lesions ranging from mild urticaria to anaphylaxis and occurs during the luteal phase. ¹ The symptoms usually occur 3–10 days before the onset of the menstrual cycle and resolve 2 days after menses has started.^1,2 Unfortunately, the diagnostic accuracy is not well studied and there are no agreed-upon diagnostic criteria for APD; hence, it is a diagnosis of exclusion.^2–4

In this article, we report a case of APD and describe the course and treatment.

Case presentation

In a family medicine clinic in a rural village in Palestine, a 30-year-old woman G2P2 + 0 had a progesterone intrauterine device (MIRENA) placed at a family planning method. Shortly after, she complained of edematous and erythematous lesions with some pruritus that disappeared 2 and 3 days later (Figure 1). The patient’s vital signs were normal. Biochemistry labs were normal with a complete blood count, IgE (immunoglobulin E), and hormone profiling. Ultrasound revealed an intrauterine device (IUD) inside the uterus with no adnexal mass or cysts. The patient mentioned similar skin manifestations in the past that appeared around her period and resolved spontaneously without treatment.

Figure 1.

Skin rash post-IUD insertion.IUD, intrauterine device.

In the beginning, corticosteroids and some antihistamines were prescribed for 1 month. Health education about medications, skincare, and red flags was discussed, and a follow-up appointment was made. The patient presented with worsened symptoms.

Reassessment and a more thorough history linked the IUD insertion and the dermatological manifestations. Dermatology and gynecology physicians were consulted, and a skin biopsy was performed. The result showed perivascular infiltration with neutrophils which raised the suspicion for APD. The IUD was removed and a follow-up was arranged. Improvement in the rash began with IUD removal. Tamoxifen was also added and the symptoms completely disappeared. The patient returned for follow-up 1 month later and all skin lesions had resolved.

Discussion

APD is a condition that can affect women of reproductive age at various points in their lives, including as early as menarche, with a higher prevalence during their third decade. ⁵ The condition presents a spectrum of symptoms, with urticaria, angioedema, dermatitis, and erythema multiforme being the most common manifestations.^5,6 These symptoms typically resolve after menopause, indicating a strong hormonal influence in triggering APD. ⁶

The pathophysiology of APD involves an aberrant immune response to progesterone, a hormone naturally produced by the body. Exposure to exogenous sources of progesterone, such as those found in oral contraceptives or IUDs, is thought to sensitize presenting cells and T helper 2 lymphocytes, leading to the production of specific IgE antibodies and a type 1 hypersensitivity reaction. ⁶ This reaction is distinct from autoimmunity, where the immune system erroneously targets and damages the body’s cells and tissues.^7–9 Autoimmune reactions are typically persistent and do not resolve solely by removing the triggering antigen source, distinguishing them from hypersensitivity reactions, which cease upon allergen removal.

In the case discussed, there is a clear distinction between an IgE-mediated hypersensitivity reaction and autoimmunity. The symptoms experienced by the patient, including erythema and pruritic lesions, resolved after the removal of the IUD and treatment with tamoxifen. This resolution supports the hypothesis of a type 1 hypersensitivity reaction, as symptoms persisted only in the presence of the allergen (progesterone from the IUD), rather than an autoimmune reaction, which would have persisted regardless of the presence or removal of the allergen source. ¹⁰

The diagnosis of APD can be challenging due to the variety of clinical presentations and the nonspecific nature of its histological features. Common findings include perivascular dermatitis with eosinophilic infiltration and interface changes, although these are not pathognomonic. ¹¹ The progesterone intradermal test, while helpful, has a high false-positive rate, which may be influenced by adverse reactions to the oil used in the test.^4,7 Nonetheless, progesterone desensitization has proven to be an effective treatment, achieving a complete response rate of over 65%.^2,3

Conclusion

The mechanism by which endogenous progesterone becomes antigenic in APD is still unknown. The pathogenicity of APD is also unclear, it is a rare disorder that lacks universal diagnostic measures and criteria, contributing to a significant delay in diagnosis. Large-scale multicenter studies are needed to develop accurate tests and establish diagnostic criteria, and APD can be treated using several methods, such as oral contraceptive pills, Gonadotropin releasing hormone (GnRH) agonists, danazol, tamoxifen, or bilateral oophorectomy.

Medical teams should be aware of this rare entity gather a thorough medical history from the patient and work collaboratively to achieve good results.

Patient perspective

The patient was satisfied and felt relaxed when the IUD was removed and tamoxifen was given without side effects. We decided to use the coitus interrupts method for family planning.