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Infantile bullous pemphigoid after meningococcal B vaccine

Infantile bullous pemphigoid after meningococcal B vaccine Dear Editor, Infantile bullous pemphigoid (BP) is a rare disease with a characteristic clinical presentation and an excellent prognosis. Its appearance has been linked to the administration of several vaccines. We describe a case of infantile BP that developed following the administration of a second dose of a meningococcal B vaccine and responded very well to treatment with oral prednisolone. A healthy 6-month-old infant developed pruritic lesions 3 days after receiving the second dose of meningococcal B vaccine. The lesions started on the palms and soles and then spread to the limbs and trunk. Examination revealed urticarial plaques and multiple tense blisters located mainly at acral sites (Fig. 1a–c). There was no mucosal involvement. Nikolsky sign was negative. Histologic examination showed subepidermal blistering with eosinophilic spongiosis and a predominantly lymphohistiocytic dermal inflammatory infiltrate with frequent eosinophils (Fig. 2a,b). Linear deposits of C3, immunoglobulin (Ig) M, and IgG were observed along the dermal-epidermal junction by direct immunofluorescence (DIF) of perilesional skin (Fig. 2c). Enzyme-linked immunoabsorbent assay (Euroimmunn) was positive for BP180 (5.69, normal value <1.01) and negative for BP230, desmoglein 1, and desmoglein 3. No maternal antibodies were detected. The boy was diagnosed with infantile BP and started on oral prednisolone at a dosage of 2 mg/kg/day. He responded very well to treatment, with complete clearance of lesions within 3 weeks and negativization of anti-BP180 antibody titers. He underwent prednisolone treatment for 7 weeks. He completed the vaccination schedule, which included a third dose of Bexsero, and has experienced no recurrences. BP is an acquired autoimmune skin disease considered to be rare in children. The diagnostic criteria for BP in infancy were established by Nemeth et al. and later simplified by Schwieger-Briel et al. to (i) urticarial plaques and blisters with an acral distribution and (ii) linear IgG and C3 deposits along the basement membrane observed by DIF (gold-standard criterion). Infantile BP (younger than 1 year) usually shows a marked involvement of palmoplantar and facial areas sparing the mucous and genital areas. Generalized lesions are more frequent than in childhood BP (older than 1 year). Urticarial lesions have been linked to infantile BP following vaccination. Most patients experience complete remission without relapse after treatment with topical or systemic corticosteroids. The pathogenesis of infantile BP is unknown, but motherto-child transmission of IgG autoantibodies has been proposed as a potential mechanism. These antibodies, however, have not to date been detected in maternal serum. Infantile BP has been linked to several triggers, including infections, autoimmune diseases, and certain drugs and vaccines such as diphtheria, tetanus, polio, hepatitis B, and meningococcal C vaccines. There has been just one previous report of infantile BP linked to meningococcal B vaccination. In