Immunobullous diseases

Canadian Consensus Statement on the Use of Intravenous Immunoglobulin Therapy in Dermatology

“…15,18 Direct immunofluorescence of perilesional skin reveals intercellular binding of IgG and/or C3 on the keratinocyte cell surface. 16,19 As antibody levels correlate with disease activity, several methods have been developed to measure circulating autoantibodies. 20 Indirect immunofluorescence (IIF), immunoblots, and enzyme-linked immunosorbent assays (ELISA) are the most commonly used techniques to quantify antibody levels.…”

“…15,16,27 Sporadic PF has a mean age at onset between 50 and 60 years and no gender predilection. 15,16,27 Fogo selvagem, a form of PF endemic to South America, typically affects younger patients, with an average onset between the ages of 20 and 30 years. 15 The disease is mediated by circulating IgG autoantibodies against desmoglein 1, a 160 kDa cadherin located in the superficial layers of the epidermis.…”

“…32 Histologically, the lesions of PF demonstrate subcorneal bullae formation with acantholysis. 15,16 Direct immunofluorescence of perilesional skin reveals intercellular binding of IgG and/ or C3 in the upper stratum malphigii. 15,16 Serologic assays establish the correlation between circulating antibodies and disease activity.…”

“…87 The clinical presentations of EBA include noninflammatory, inflammatory, and mucous membrane-dominant forms. 16,27,[85][86][87] Many patients with EBA have increased skin fragility, with blisters occurring at sites of trauma. 16,27,[85][86][87] Tense serous or hemorrhagic blisters typically develop on the extensor surfaces of extremities, elbows, knees, fingers, and toes.…”

“…16,27,[85][86][87] Many patients with EBA have increased skin fragility, with blisters occurring at sites of trauma. 16,27,[85][86][87] Tense serous or hemorrhagic blisters typically develop on the extensor surfaces of extremities, elbows, knees, fingers, and toes. 16,27,[85][86][87] The lesions often heal with scarring or milia formation and may be associated with nail dystrophy and scarring alopecia.…”