What isautoimmuneprogesteronedermatitis?
Autoimmune progesterone dermatitis is a rare,cyclicalmucocutaneoushypersensitivityreaction to peak levels ofendogenousprogesterone seen in thelutealphase of the menstrual cycle.
Who gets autoimmune progesterone dermatitis?
Autoimmune progesterone dermatitis is predominantly a condition of young adult women with ameanage of onset of 27.3 years; it has also been reported in adolescents after themenarcheand in olderpremenopausalwomen. It often, but not always, occurs in women who have previously receivedexogenousprogestogens, such as theoral contraceptive pillor fertility treatments. It may present in pregnancy or the post-partum period. There is nogeneticrisk.
What causes autoimmune progesterone dermatitis?
The cause of autoimmune progesterone dermatitis is not known. Hypotheses include:
- Exogenous progestogens may trigger a type 1 (immediate) hypersensitivity reaction with the formation of progesterone-specificimmunoglobulinE (IgE)antibodiesand amast cell-mediated response, which may target progesterone receptors expressed above thebasallayeronkeratinocytes.
- There may be a type 4 (delayed) hypersensitivity reaction to progestogens.
Sensitised patients then have cyclical symptoms due to an ongoing autoimmune response to the elevated levels of progesterone seen in the luteal phase of the menstrual cycle.
The cause in patients with no prior exposure to exogenous progestogens is unclear.
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What are the clinical features of autoimmune progesterone dermatitis?
Autoimmune progesterone dermatitis characteristically presents as arashthat appears 3–4 days before menstruation when progesterone levels peak. The rash resolves within a few days after the onset of menstruation as progesterone levels reduce, only torecurjust before the next period. This cyclical pattern may not be apparent in women with irregular menses.
The type of rash seen in autoimmune progesterone dermatitis can be variable, but the majority of patients present with:
Other reportederuptionsinclude:
- Non-specific skin changes —morbilliformrash,丘疹andplaques,vesiculobullousand vesiculopustularlesions,petechiae, andpurpura
- Fixed drugeruption
- Cyclicvulvovaginitis
- Stomatitis
- Erythemamultiforme
- Annularerythema
- Necrolytic migratory erythema–like eruption.
Skin changes typically affect the trunk and limbs but may involve the face, oralmucosa, lips, andgenitalia.
Autoimmune progesterone dermatitis
What are the complications of autoimmune progesterone dermatitis?
- Spontaneous abortion has been reported
How is autoimmune progesterone dermatitis diagnosed?
Autoimmune progesterone dermatitis should be considered if:
- There is a consistent and recurringpremenstrualflare of a rash
- The rash is reproducible followingintramuscularprogesterone injection — oral orintravaginalchallenge tests may be alternatives
- The rash can be prevented by suppression of ovulation.
Intradermaltests with progesterone can be performed and may trigger local orsystemicreactions. However, suitablereagentsmay be difficult to source, the tests are not standardised, andirritantreactions may occur.
Other tests not routinely available includelymphocytetransformation tests, interferon gamma release assay, and demonstration ofprogesterone-specific IgE on ELISA.
There are no diagnosticskinbiopsyfindings that distinguish disease triggered by progesterone from otheraetiologies.
What is thedifferential diagnosisfor autoimmune progesterone dermatitis?
- Drug eruption
- Spontaneous urticaria
- Atopicdermatitisor otherdermatosesthat can flare premenstrually
- Oestrogendermatitis
What is the treatment for autoimmune progesterone dermatitis?
General measures
Specific treatments
- Oral contraceptive pill
- Gonadotrophin releasing hormones
- Tamoxifen
- Oophorectomy
- Progesteronedesensitisationby injection or intravaginal topical application
What is the outcome for autoimmune progesterone dermatitis?
Autoimmune progesterone dermatitis continues unabated without treatment. Response to treatment is variable. Some women experience a reduction in symptoms during pregnancy which is thought to be due to a natural desensitisation process that occurs with the gradual increase in progesterone during pregnancy; others experience a worsening of symptoms during pregnancy. Autoimmune progesterone dermatitis resolves at menopause and progesterone-containing hormone replacement therapy should not be prescribed.