Open AccessBrunsting-Perry Type Pemphigoid Causing Secondary Cicatricial Alopecia in 2 Patients
Author(s) -
Maria Fernanda Reis Gavazzoni Dias,
Enoï Aparecida Guedes Vilar,
Catherine Bento,
Raquel Barreto Ibanez,
Aline Falci Loures,
Maria Barreto Oliveira Campos,
Júlia Monaco Santos
Publication year - 2018
Publication title -
skin appendage disorders
Language(s) - Uncategorized
Resource type - Journals
SCImago Journal Rank - 0.773
H-Index - 13
eISSN - 2296-9195
pISSN - 2296-9160
DOI - 10.1159/000485570
Subject(s) - cicatricial pemphigoid , medicine , epidermolysis bullosa acquisita , dermatology , bullous pemphigoid , scalp , direct fluorescent antibody , pemphigoid , pathology , antigen , antibody , immunology
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.