Subject(s)
Anti-Infective Agents/therapeutic use , Dapsone/therapeutic use , Pemphigus/drug therapy , Pemphigus/immunology , Acitretin/therapeutic use , Adalimumab , Anti-Bacterial Agents/therapeutic use , Anti-Inflammatory Agents/therapeutic use , Antibodies, Monoclonal, Humanized/therapeutic use , Colchicine/therapeutic use , Drug Therapy, Combination , Humans , Immunoglobulin A , Keratolytic Agents/therapeutic use , Prednisone/therapeutic use , Tetracycline/therapeutic use , Tubulin Modulators/therapeutic useABSTRACT
OBJECTIVES: To evaluate the reactivity of indirect immunofluorescence using rat bladder epithelium as a substrate in patients with pemphigus foliaceus and pemphigus vulgaris from the Department of Dermatology, University of São Paulo Medical School, Brazil. METHODS: Thirty-two patients (8 male and 24 female) from the Department of Dermatology, University of São Paulo Medical School, were selected. Three had mucosal pemphigus vulgaris, 20 had mucocutaneous pemphigus vulgaris, and 9 had pemphigus foliaceus. Patients' sera were tested by indirect immunofluorescence performed on human foreskin and rat bladder epithelium and by ELISA assays utilizing baculovirus-expressed recombinant desmoglein 3 and desmoglein 1. RESULTS: No patients with mucosal pemphigus vulgaris, 5 of 20 patients with mucocutaneous pemphigus vulgaris (25%) and 4 of 9 patients with pemphigus foliaceus (44%) had positive indirect immunofluorescence using rat bladder epithelium as a substrate. CONCLUSION: Indirect immunofluorescence using rat bladder epithelium as a substrate is recommended whenever a diagnosis of paraneoplastic pemphigus is considered. The identification of a subset of pemphigus foliaceus and pemphigus vulgaris patients that recognizes desmoplakins by this laboratory tool is critical to avoid the misdiagnosis of paraneoplastic pemphigus.
Subject(s)
Autoantibodies/immunology , Desmoglein 1/immunology , Desmoglein 3/immunology , Fluorescent Antibody Technique, Indirect/methods , Pemphigus/immunology , Urinary Bladder/immunology , Adult , Aged , Animals , Enzyme-Linked Immunosorbent Assay , Female , Humans , Male , Middle Aged , Pemphigus/pathology , Rats , Sensitivity and Specificity , Urinary Bladder/cytology , Urothelium/immunologyABSTRACT
OBJECTIVES: To evaluate the reactivity of indirect immunofluorescence using rat bladder epithelium as a substrate in patients with pemphigus foliaceus and pemphigus vulgaris from the Department of Dermatology, University of São Paulo Medical School, Brazil. METHODS: Thirty-two patients (8 male and 24 female) from the Department of Dermatology, University of São Paulo Medical School, were selected. Three had mucosal pemphigus vulgaris, 20 had mucocutaneous pemphigus vulgaris, and 9 had pemphigus foliaceus. Patients’ sera were tested by indirect immunofluorescence performed on human foreskin and rat bladder epithelium and by ELISA assays utilizing baculovirus-expressed recombinant desmoglein 3 and desmoglein 1. RESULTS: No patients with mucosal pemphigus vulgaris, 5 of 20 patients with mucocutaneous pemphigus vulgaris (25 percent) and 4 of 9 patients with pemphigus foliaceus (44 percent) had positive indirect immunofluorescence using rat bladder epithelium as a substrate. CONCLUSION: Indirect immunofluorescence using rat bladder epithelium as a substrate is recommended whenever a diagnosis of paraneoplastic pemphigus is considered. The identification of a subset of pemphigus foliaceus and pemphigus vulgaris patients that recognizes desmoplakins by this laboratory tool is critical to avoid the misdiagnosis of paraneoplastic pemphigus.
Subject(s)
Adult , Aged , Animals , Female , Humans , Male , Middle Aged , Rats , Autoantibodies/immunology , Desmoglein 1/immunology , /immunology , Fluorescent Antibody Technique, Indirect/methods , Pemphigus/immunology , Urinary Bladder/immunology , Enzyme-Linked Immunosorbent Assay , Pemphigus/pathology , Sensitivity and Specificity , Urinary Bladder/cytology , Urothelium/immunologyABSTRACT
Chediak-Higashi syndrome (CHS), typically presents with partial albinism and severe hematological abnormalities. About 10% of the patients have a mild adult form associated with various neurological manifestations. We describe the case of a 24-year-old woman with parkinsonism that responded well to antiparkinsonian drugs.
