Long-lasting localized pemphigus vulgaris without detectable serum autoantibodies against desmoglein 3 and desmoglein 1

• Published in: Indian journal of dermatology Vol. 61; no. 4; pp. 427 - 429
• Main Authors: Yoshifuku, Asuka, Fujii, Kazuyasu, Kawahira, Hisao, Katsue, Hiromi, Baba, Atsunori, Higashi, Yuko, Aoyama, Yumi, Kanekura, Takuro
• Format: Journal Article
• Language: English
• Published: India Wolters Kluwer - Medknow Publications 01.07.2016; Medknow Publications and Media Pvt. Ltd; Medknow Publications & Media Pvt. Ltd; Medknow Publications & Media Pvt Ltd; Wolters Kluwer Medknow Publications
• Subjects: Anti-desmoglein 1 and 3 antibodies; Autoantibodies; Cadherins; Case Report; Case studies; Diagnosis; direct immunofluorescence test; Health aspects; indirect immunofluorescence test; localized; Pemphigus; pemphigus vulgaris; Risk factors; India; direct immunofluorescence test; localized; pemphigus vulgaris; indirect immunofluorescence test; Anti-desmoglein 1 and 3 antibodies
• ISSN: 0019-5154; 1998-3611
• DOI: 10.4103/0019-5154.185712

Pemphigus vulgaris (PV) is an autoimmune blistering disease elicited by anti-desmoglein (DsG) 3 antibody. Although skin lesions tend to be distributed over the entire body, in some patients, they are confined to a restricted area. We report two patients who presented with long-lasting localized PV without detectable anti-DsG antibodies after suffering antibody-positive systemic PV. Initial treatment with prednisolone (PSL) was successful in both patients, but a local relapse occurred on the cheek or lower lip after a reduction in the PSL dose. Biopsy of the localized lesions showed suprabasal acantholysis; no serum DsG antibodies were found. Local immunosuppression therapy was effective in both patients. Based on our findings, we suggest that localized PV without detectable antibodies can develop after systemic PV.