It was interesting to read in the March edition of the journal Pediatric Dermatology of a little known entity called autoimmune progesterone dermatitis.
[Aghazadeh N, Chattha A, Hartz M et al. Autoimmune progesterone dermatitis in the adolescent population. Pediatr Dermatol 2021 ;38(2) :380-384.]
What is Autoimmune Progesterone Dermatitis?
It is a rare, cyclical skin condition which is due to a surge in the patient’s own progesterone hormone production (so called endogenous source) at a certain point in the menstrual cycle.
Why does it happen?
We are still not completely sure but it is thought that in affected women there is an immune reaction to the progesterone hormone surge during the luteal phase of the menstrual cycle.
[Özdemir Ö, Yahsi G, Atalay C. Autoimmune Progesterone Dermatitis: A Case Report. Rev Bras Ginecol Obstet 2019;41:203-205.]
There was some thinking that previous progesterone exposure eg from the oral contraceptive pill, progesterone releasing intra-uterine devices or depots, pregnancy, (ie exogenous exposure) can stimulate a hypersensitivity reaction to endogenous hormones. However, there have been cases reported as young as 12 years of age who had no prior exposure to exogenous progesterone.
[DeRosa I, Bender B, Centilli M. Autoimmune Progesterone Dermatitis. Cutis 2018; 102(4): E12-E14.]
When does it happen?
The skin eruption typically occurs 3-4 days before the onset of the menstrual period and resolves 2-3 days after the onset of the menstrual period.
What does it look like?
The presentation is surprisingly varied and hence why the diagnosis can, so often, be delayed. It can look like eczema (red, dry itchy), urticaria (hives), erythema multiforme-like (look like targets), or angioedema-like (large skin swellings).
Some can present only mildly or some can present very floridly with a presentation that might progress to anaphylaxis.
The diagnosis can also be hard to make if someone does not have regular periods.
How is the diagnosis made?
Three things need to be present to make the diagnosis:
- The presence of skin lesions/rashes in the luteal phase of the menstrual cycle,
- A positive response to intradermal (into the skin) injection of progesterone, and
- Symptomatic improvement in the skin rashes after inhibiting progesterone secretion by suppressing ovulation.
[Apurwa A, Patil S, Chaudhari P et al. Autoimmune progesterone dermatitis. Indian J Dermatol. 2018 ;63(2) :188-190.]
What is the treatment?
It is basically suppression of the internal hormonal progesterone surge. This is normally achieved by taking the oral contraceptive pill which suppresses ovulation.
Other drugs can be given in more difficult cases, for example gonadotrophin-hormone releasing agonists, danazol, omalizumab (used in refractory urticaria) or even progesterone desensitisation. In some severe cases, removal of the ovaries is even needed.
The authors’ conclusions?
Autoimmune progesterone dermatitis is rare or underreported in women. It is even more rarely reported in adolescents and there is a significant delay in making the diagnosis. It basically needs better definition to aid diagnosis and treatment of this under recognised condition.
Kind regards,
Sandy
Dr Sandy Flann, Consultant Dermatologist
