Pemphigus vegetans mit autoantikorpern gegen rekombinantes desmoglein 3

Pemphigus vegetans is a rare variant of pemphigus vulgaris and belongs to the group of autoimmune blistering diseases. A 73-year old female patient suffering from pemphigus vulgaris presented with erosions on oral and laryngeal mucous membranes. With systemic treatment the mucous membrane lesions improved, but a vegetating erosive plaque developed on the left inguinal region. A biopsy from this site showed acanthosis and suprabasal acantholytic blister formation. Direct immunofluorescence microscopy revealed an epidermal intercellular deposition of IgG antibodies. Indirect immunofluorescence analysis using monkey oesophagus as a substrate demonstrated the presence of circulating IgG autoantibodies binding to the epithelium with an intercellular staining pattern and a titer of 1:320. By ELISA, using recombinant forms of desmoglein 1 and 3, the serum of our patient showed high levels of autoantibodies to desmoglein 3, but not to desmoglein 1. Systemic treatment with a intravenous pulse therapy of dexamethasone and cyclophosphamide as well as intralesional injections of the left inguinal region led to complete remission of the skin lesions. This report confirms the close relationship between pemphigus vulgaris and pemphigus vegetans. With regard to this case, we discuss clinical spectrum, diagnosis and therapeutic options of pemphigus vegetans. To the best of our knowledge, this is the first case of pemphigus vegetans in which the autoimmune response has been characterized using recombinant forms of desmoglein 1 and 3.