Three case reports of post immunization and post viral Bullous Pemphigoid: looking for the right trigger

Abstract

BackgroundBullous pemphigoid (BP) is a blistering skin disorder infrequent in infancy and rarely reported in medical literature.Case PresentationHere we describe three cases of BP which were referred to our department in the last 15 years. Two of them developed an eruption of bullous lesions just a few days after vaccination for diphtheria, tetanus, pertussis, poliomyelitis, hepatitis B and Haemophilus influenzae B. The third patient developed the same blistering lesions shortly after herpetic stomatitis. In all three cases, clinical diagnosis was confirmed by histological examination which showed subepidermal bullae with a dermal inflammatory infiltrate, and direct immunofluorescence of perilesional skin showed linear IgG and C3 deposits along the basement membrane zone. Immunoblot assay was positive for BP antigen 180. Treatment with oral prednisone was instituted and the lesions resolved in two out of three patients; the third one was treated with an immunosuppressive agent (tacrolimus) and corticosteroid and subsequently with intravenous immunoglobulin and plasmapheresis, due to an underlying complex autoimmune disease.ConclusionAlthough the mechanism of induction of BP is still unclear, the close relationship between trigger events (immunization or viral infection) and onset of the disease arises a possible association.

Should SARS‐CoV‐2 influence immunosuppressive therapy for autoimmune blistering diseases?

“…In particular, we asked whether patients in immunosuppressive therapy Among possible trigger factors of autoimmune blistering diseases, immunization and viral infections are mentioned in the literature, although the underlying immunological mechanism is still unclear. [7][8][9] The most acceptable hypothesis involves the possible molecular mimicry existing between viral and epidermal proteins, and over activation of the immune system as a consequence of the viral attack. 9 Indeed, in autoimmune blistering disorders, once the autoantibodies bind to…”

Skin Barrier and Autoimmunity—Mechanisms and Novel Therapeutic Approaches for Autoimmune Blistering Diseases of the Skin

“…Cell damage has been proposed as a common “triggering factor” which causes development of pathogenic adaptive autoimmune reactions—cell damage due to surgical trauma (23), UV radiation (24), neurological disorders and other pre-existing conditions (25–29), viral infection (30–33), and radiotherapy (34–36) have all been associated with disrupted skin barrier function and development of AIBDs (37). Cell damage via necrosis or necroptosis releases a complex intracellular milieu into the extracellular space which serves as a source of sensitizing autoantigens (38); additionally cell death results in the release of damage associated molecular patterns which stimulate localized inflammation and wound healing processes (39, 40).…”

Terapi Dapson Pada Pemfigoid Bulosa

“…Membran mukosa dapat terkena pada 10-35% kasus. 3,4 Pada penyakit ini terdapat produksi autoantibodi yang terikat pada protein transmembran (BP180) dan plak hemidesmosom (BP230), yang akan mengaktivasi sistem komplemen, perekrutan sel inflamasi (terutama eosinofil dan neutrofil) dan pembentukan bula subepidermis. Deposit IgG linier dan C3 pada basal membrane zone dapat diamati dengan pemeriksaan imunofluoresensi langsung atau Direct Immunofluorescence (DIF).…”