The chancre of pemphigus on the scalp as the first symptom of mucosal-dominant pemphigus vulgaris in an elderly man taking ramipril.

Pemphigus vulgaris (PV) is a life-threatening, autoimmune blistering disease of the mucocutaneous surfaces. Intraepidermal suprabasilar blistering and acantholysis in PV is caused by bound and circulating IgG autoantibodies against keratinocyte cell-surface antigens, namely desmosomal desmoglein 3 and desmoglein 1. A patient taking ramipril with chancre of pemphigus, i.e. single initial persistent lesion, which in this patient was localized on the scalp and heralded further PV mucosal lesions for 8 months is described. Direct immunofluorescence on perilesional skin revealed IgG(+), IgG1(+/-), IgG4(++) and C3(+/-) pemphigus deposits in the follicular and extrafollicular epithelium. Indirect immunofluorescence on monkey oesophagus revealed IgG and IgG4 antibodies directed against desmosomal proteins of keratinocytes. ELISA demonstrated elevated serum levels of IgG autoantibodies directed against desmoglein 3, but not desmoglein 1. Both the symptoms and progression of mucosal dominant PV may vary widely. Controlled trials on the treatment of autoimmune blistering dermatoses are still scarce. Individual approach in identifying and subsequent elimination of the plausible triggering or/and disease sustaining factors should be a common practice. [ABSTRACT FROM AUTHOR]