| Literature DB >> 30410903 |
Maria Fernanda Reis Gavazzoni Dias1, Enöi Aparecida Guedes Vilar1, Camila de Oliveira Bento1, Raquel Barreto Ibanez1, Aline Falci Loures1, Maria Barreto Oliveira Campos1, Júlia Monaco Santos1.
Abstract
Brunsting-Perry type pemphigoid (BPP) is a rare subepidermal blistering disease and a cause of secondary cicatricial alopecia. It was originally described by Brunsting and Perry in 1957 as a rare variant of cicatricial pemphigoid, characterized by bullous lesions limited to the head, neck, scalp, and upper trunk with mild or no mucosal involvement. We report 2 cases of BPP cicatricial alopecia with histopathology of subepidermal blister formation, different clinical presentation, and different salt-split test results. One patient had features of bullous pemphigoid (BP) with important oral mucosal involvement (not yet reported in the literature), and the second patient had typical features of epidermolysis bullosa acquisita (EBA). The secondary cicatricial alopecia may be due to different antigens associated with either BP or EBA. The phenomenon of epitope spreading could explain the association between 2 distinctive bullous diseases in the same patient, justifying the divergent findings of the immunofluorescence. The specific target antigen of BPP is yet to be defined.Entities:
Keywords: Alopecia; Brunsting-Perry type pemphigoid; Pemphigoid
Year: 2018 PMID: 30410903 PMCID: PMC6219224 DOI: 10.1159/000485570
Source DB: PubMed Journal: Skin Appendage Disord ISSN: 2296-9160