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Disease Profile

Progestogen hypersensitivity

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

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ICD-10

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Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

APD; autoimmune progesterone dermatitis

Categories

Skin Diseases

Summary

Progestogen hypersensitivity causes a skin reaction that typically occurs during a woman's menstrual cycle. Symptoms usually begin 3-10 days before a woman's period and go away when her period is over. Skin symptoms may include rash, swelling, itching, hives, and red, flaky patches. More severe symptoms can include open sores, wheezing, and an asthma-like reaction. Progestogen hypersensitivity symptoms stop at the time of menopause. The exact cause of progestogen hypersensitivity is unknown, but many women with this condition have had exposure to external sources of progesterone such as oral contraceptives or drugs used for fertility treatments. Some women develop progestogen hypersensitivity in response to the progesterone naturally produced by the body. Diagnosis is based on the symptoms, clinical exam, and a skin test. Treatment is focused on managing the symptoms and may include medications that block the activity of progesterone or stop the body from making progesterone. Progesterone desensitization and surgery to remove the ovaries may cure the symptoms of progestogen hypersensitivity.[1][2][3][4][5]

Symptoms

The following list includes the most common signs and symptoms in women with progestogen hypersensitivity. These features may be different from person to person. Some people may have more symptoms than others, and they can range from mild to severe. This list does not include every symptom that has been described in the condition.

Symptoms of progestogen hypersensitivity include:[1][2][5] 

The symptoms of progestogen hypersensitivity usually begin in the late 20s. Symptoms first appear a few days before a woman starts her period and go away after her period is over. Rarely, progestogen hypersensitivity may cause respiratory symptoms such wheezing and difficulty breathing. Progestogen hypersensitivity ends after a woman stops having periods (menopause).[1][2]

Cause

The exact cause of progestogen hypersensitivity is not known. Many women with progestogen hypersensitivity have had exposure to an external source of progesterone such as oral contraceptives or drugs used in fertility treatments. However, some women develop progestogen hypersensitivity in response to the progesterone made naturally by the body.[1][2][5]

Diagnosis

The diagnosis of progestogen hypersensitivity is based on the symptoms, clinical exam, and a skin test to look for evidence of a skin reaction to progesterone.[4][5]

Treatment

Treatment of progestogen hypersensitivity is focused on managing the symptoms. Treatment typically involves the use of medications that block the action of progesterone or stop it from being made by the body. Medications may include oral contraceptives, steroids, tamoxifen, and gonadotropin-releasing hormones. Some women have been treated with progesterone desensitization. For severe cases, surgical removal of ovaries (an oophorectomy) is curative.[4][5]

Specialists involved in the care of someone with progestogen insensitivity may include:

  • Obstetrician/gynecologist
  • Endocrinologist
  • Dermatologist

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

    Learn more

    These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

    Where to Start

    • DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.

      In-Depth Information

      • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
      • PubMed is a searchable database of medical literature and lists journal articles that discuss Progestogen hypersensitivity. Click on the link to view a sample search on this topic.

        References

        1. Li RC, Buchheit KM, Bernstein JA. Progestogen Hypersensitivity. Curr Allergy Asthma Rep. Jan 19, 2018; 18(1):1. https://pubmed.ncbi.nlm.nih.gov/29349660/.
        2. Foer D, Buchheit KM. Presentation and natural history of progestogen hypersensitivity. Ann Allergy Asthma Immunol. Feb 2019; 122(2):156-159. https://pubmed.ncbi.nlm.nih.gov/30712576.
        3. Nguyen T, Razzaque Ahmed A. Autoimmune progesterone dermatitis: Update and insights. Autoimmun Rev. Feb 2016; 15(2):191-7. https://pubmed.ncbi.nlm.nih.gov/26554933.
        4. Foer D, Buchheit KM. Progestogen Hypersensitivity: An Evidence-Based Approach to Diagnosis and Management in Clinical Practice. Immunol Allergy Clin North Am. Nov 2017; 37(4):773-784. https://pubmed.ncbi.nlm.nih.gov/28965640/.
        5. Buchheit KM, Bernstein JA. Progestogen hypersensitivity. UpToDate. Updated Sept 22, 2020; https://www.uptodate.com/contents/progestogen-hypersensitivity.

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