Bullous Pemphigoid Associated With COVID-19 Vaccines: An Italian Multicentre Study

Abstract

Bullous pemphigoid (BP) is an autoimmune bullous disease caused by circulating autoantibodies toward the hemidesmosomal antigens BP180 and BP230. Cases of BP have been described following vaccinations against tetanus, poliomyelitis, diphtheria, influenza, pneumococcus, meningococcus, hepatitis B and rabies. The putative mechanism by which COVID-19-vaccines may induce BP has not been clarified. An Italian multicentre study was conducted to collect clinical, histopathological and immunopathological data of patients with BP associated with COVID-19-vaccines. Twenty-one cases were collected, including 9 females and 12 males (M/F = 1.3) with a median age at diagnosis of 82 years. Seventeen patients received the COMIRNATY Pfizer-BioNTech vaccine, two the Moderna mRNA-1273 vaccine, one the ChAdOx1/nCoV-19-AstraZeneca/ Vaxzevria vaccine and one received the first dose with the ChAdOx1/nCoV-19-AstraZeneca/Vaxzevria vaccine and the second dose with the COMIRNATY Pfizer-BioNTech vaccine. Median latency time between the first dose of anti-SARS-CoV-2 vaccine and the onset of cutaneous manifestations was 27 days. Median BPDAI at onset was 42. Eleven out of seventeen patients (65%) had positive titres for anti-BP180 antibodies with a median value of 106.3 U/mL on ELISA; in contrast, only five out of seventeen (29%) were positive for anti-BP230 antibodies, with a median of 35.3 U/mL. In conclusion, in terms of mean age, disease severity at diagnosis and clinical phenotype vaccine-associated BP patients seem to be similar to idiopathic BP with an overall benign course with appropriate treatment. On the other hand, the slight male predominance and the reduced humoral response to BP230 represent peculiar features of this subset of patients.

A Patient Develops Bullous Rash After Receiving the Second Dose of COVID-19 Vaccine

“…The mean age of the patients was 79 years and there was a predominance of males over females. The onset of disease was reported both after the first and second dose with a similar latency period, however, recurrence of disease was uncommon [8]. Our patient received his booster dose eight months later and had an uneventful post-vaccination course.…”

“…Our patient received his booster dose eight months later and had an uneventful post-vaccination course. All patients were treated with topical and/or systemic corticosteroids, with or without the addition of immunosuppressive drugs, with a good clinical response in every case [8].…”

Five cases of new‐onset pemphigus following vaccinations against coronavirus disease 2019

“… 9 The role of personal susceptibility is even more supported by the fact that autoimmune bullous diseases with higher incidence in their idiopathic form, such as bullous pemphigoid, also seem to be more frequently triggered or boosted by COVID‐19 vaccines. 10 In addition, Case 1 suggests that chronic immunosuppressive therapies prescribed for other concomitant diseases might maintain the autoimmune bullous disorder in a latent or subclinical stage until the balance is broken by strong precipitating factors such as vaccinations.…”

Exacerbation of Autoimmune Bullous Diseases After Severe Acute Respiratory Syndrome Coronavirus 2 Vaccination: Is There Any Association?

“…They reported similar clinical presentations, with a predominance of male patients. Immunopathological features were typical, but anti-BP230 autoantibody was remarkably reduced ( 22 ). It should be noted that some authors argue against the casualty relation between the SARS-CoV-2 vaccine and AIBDs as they recorded no increase in disease incidence in the year of vaccination ( 23 ).…”