Pemphigus

Abstract

Pemphigus is a rare autoimmune blistering disorder involving the skin and mucosae. Three types of pemphigus have been described, including pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus. Diagnosis of the type of pemphigus is made by the combination of clinical features, histological and direct immunofluorescence findings, and serum analysis by indirect immunofluorescence, immunoblot analysis and enzyme-linked-immunosorbent-assay (Elisa). Most patients are treated with oral corticosteroids, sometimes associated with immunosuppressants. Patients with treatment failure or with contraindications to systemic corticosteroids and/or immunosuppressants may be treated with intravenous immunoglobulins or rituximab, an anti-CD20 monoclonal antibody.

Diagnostic Value of Anti-Desmoglein 1 and 3 Antibodies and Anti-BP180 and 230 Antibodies in Autoimmune Bullous Dermatoses

“…The IIF method in patients with bullous pemphigoid can detect the presence of hemidesmosomal antibodies against the epidermal basement membrane in the patient's serum. The ELISA method can detect specific anti-BP180 and anti-BP230 antibodies, and it is found that the serum of more than 90% of bullous pemphigoid patients contains anti-BP180 antibodies, which is related to the disease severity [3][4][5] . Previous literature shows that direct immunofluorescence (DIF) is the "gold standard" for diagnosing bullous dermatoses, but there are false negatives or false positives in clinical practice [3] .…”

“…The ELISA method can detect specific anti-BP180 and anti-BP230 antibodies, and it is found that the serum of more than 90% of bullous pemphigoid patients contains anti-BP180 antibodies, which is related to the disease severity [3][4][5] . Previous literature shows that direct immunofluorescence (DIF) is the "gold standard" for diagnosing bullous dermatoses, but there are false negatives or false positives in clinical practice [3] . To explore the clinical application and significance of the detection of anti-Dsg1, anti-Dsg3, anti-BP180, and anti-BP230 antibodies in the serological diagnosis of bullous dermatoses, the IIF and ELISA test results of patients with bullous dermatoses in Dalian were collected and analyzed, providing the basis for the diagnosis and treatment of pemphigus and bullous pemphigoid.…”