Marie Antoinette Syndrome Following COVID-19 Vaccination

Marie Antoinette syndrome refers to a condition in which scalp hair suddenly turns white. The name alludes to the unhappy Queen Marie Antoinette of France, whose hair allegedly turned white the night before her last walk to the guillotine during the French Revolution. Now it is known that this syndrome refers to diffuse alopecia areata occurring in a patient. Recently a case report of such a syndrome occurring after COVID vaccination was published in the International Journal of Dermatology.

A 39-year-old Caucasian presented with a sudden greying of the hair, which was rapidly followed by diffuse hair loss. The process started 4 days after the first dose of Pfizer/BioNTech BTN162b2 vaccine. The patient did not have a history of pre-existing AA or other autoimmune conditions. Physical examination showed a widespread hair loss in the central area of the scalp and at the vertex along with nail pitting. After the second vaccine dose, the alopecic areata considerably enlarged. Clinical presentation was consistent with a diagnosis of Marie Antoinette syndrome, a rare variant of alopecia areata.

The patient was treated with a tapering course of oral prednisone and topical clobetasol 0.05% foam. After 4 weeks of treatment, trichoscopy showed a few exclamation mark hairs and yellow dots on a background of regrowing hair, which also displayed an initial restoring of the original hair pigmentation. After 6 weeks of therapy, the patient showed almost complete regrowth with partial repigmentation of the hairs (Severity of Alopecia Tool [SALT] score pre-treatment: 46.2%; SALT score after treatment: 5.2%). No recurrence was observed after 6 months of follow-up.

In this patient, the following features supported the vaccine as a trigger for alopecia areata:

• Short latency time between the onset of AA and vaccination
• Disease worsening following the second vaccine dose
• Prompt recovery after therapy

After a thorough review of the PubMed literature, a total of 14 patients have been diagnosed with alopecia areata following COVID-19 vaccines. A female predominance and a mean age of 42.8 years (range 15–80 years) was noted in the cases. Eleven out of 14 cases were reported following mRNA vaccines while only three were linked to ChAdOx1 nCoV-19 vaccine (Oxford/AstraZeneca). Personal or family history of AA was considered as an important risk factor and was present in eight patients. The latency time between vaccine administration and onset of alopecia areata ranged from a few days to 4 months.

In 10 cases, hair loss was described as "widespread" and "rapidly progressive"; two of these were diagnosed with alopecia universalis. Almost all patients demonstrated improvement after different treatment modalities, including tofacitinib. This was the first case showing the peculiar features of Marie Antoinette syndrome, a rare variant of AA characterized by a sudden ("overnight") greying of the scalp hair presumably due to a preferential loss of pigmented hair post COVID vaccination.

To conclude, alopecia areata occurs most frequently after mRNA vaccines against SARS-CoV-2; it presents mostly with widespread hair loss rather than classic patches.

References

Baffa ME, Pipitò C, Montefusco F, Senatore S, Brandini L, Antiga E, Maglie R. Marie Antoinette syndrome following COVID-19 vaccination. Int J Dermatol. 2022 Jul;61(7):903-904. doi: 10.1111/ijd.16262. Epub 2022 May 14. PMID: 35567509.