Assuntos
Surdez , Perda Auditiva Neurossensorial , Ceratodermia Palmar e Plantar , Conexinas/genética , Surdez/complicações , Surdez/genética , Perda Auditiva Neurossensorial/genética , Humanos , Ceratodermia Palmar e Plantar/complicações , Ceratodermia Palmar e Plantar/genética , Mutação , LinhagemAssuntos
Autoanticorpos/sangue , Autoantígenos/imunologia , Laminina/imunologia , Dermatose Linear Bolhosa por IgA/sangue , Colágenos não Fibrilares/imunologia , Idoso de 80 Anos ou mais , Humanos , Imunoglobulina A/sangue , Imunoglobulina G/sangue , Dermatose Linear Bolhosa por IgA/patologia , Masculino , Colágeno Tipo XVIIAssuntos
Reações Antígeno-Anticorpo , Autoanticorpos/sangue , Técnicas Imunoenzimáticas/métodos , Pênfigo/diagnóstico , Adulto , Idoso , Autoanticorpos/imunologia , Autoantígenos/imunologia , Desmogleína 1/imunologia , Desmogleína 3/imunologia , Reações Falso-Negativas , Feminino , Humanos , Pessoa de Meia-Idade , Pênfigo/sangue , Pênfigo/imunologia , Fatores de TempoRESUMO
BACKGROUND: Bullous pemphigoid (BP) is the most common autoimmune blistering disease. BP180 is the primary autoantigen of BP, and in a portion of BP cases, BP230 is the only target of autoantibodies. Such BP is called BP230-type BP. BP230-type BP tends to show milder clinical phenotypes than conventional BP, but the reason is unclear. The pathogenic roles of autoantibodies and complement activation have been shown in conventional BP, but the distribution of IgG subclasses and the degree of complement deposition in BP230-type BP remain unclear. OBJECTIVE: To compare the distribution of IgG subclasses and the degree of complement deposition in BP230-type BP with those in conventional BP with autoantibodies to BP180 and BP230 (BP180-BP230-type BP). METHODS: The diagnosis of BP was confirmed by the histopathology of the lesions, the deposition of IgG and complement in the perilesional skin and the presence of circulating autoantibodies to BP180 and BP230. The disease severity was determined by bullous pemphigoid disease area index. The deposition of IgG subclasses and complement deposition were examined by direct immunofluorescence of the perilesional skin in 6 BP230-type BP cases and 11 BP180-BP230-type BP cases. RESULTS: Sixty seven percent of BP230-type BP cases show a mild clinical phenotype. All BP230-type BP cases and 82% of BP180-BP230-type BP cases were found to demonstrate the clear deposition of IgG4 at the basement membrane zone of skin specimens. Notably, the deposition of IgG1 and IgG3 was faint or negative in all of the BP230-type BP cases, whereas they were clearly detected in 91% and 64% of the BP180-BP230-type BP cases, respectively. The deposition of complement C3 tended to be weaker in BP230-type BP than in BP180-BP230-type BP. CONCLUSION: The mild clinical phenotype of BP230-type BP may correlate with the weaker deposition of IgG1, IgG3 and complement in the skin lesions.
Assuntos
Autoanticorpos/sangue , Autoantígenos/imunologia , Complemento C3/metabolismo , Distonina/imunologia , Imunoglobulina G/metabolismo , Colágenos não Fibrilares/imunologia , Penfigoide Bolhoso/metabolismo , Adulto , Idoso , Idoso de 80 Anos ou mais , Membrana Basal/metabolismo , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Penfigoide Bolhoso/sangue , Fenótipo , Índice de Gravidade de Doença , Pele/metabolismo , Colágeno Tipo XVIIRESUMO
BACKGROUND: More than half of patients with pemphigus experience relapse during the disease course. The risk factors and clinical and immunological characteristics of relapse remain largely unclear. OBJECTIVES: To elucidate the risk factors and clinical features of pemphigus relapse. METHODS: We carried out a retrospective review of the clinical records of 42 cases of pemphigus at a single centre. RESULTS: Sixty-two per cent of patients experienced relapse, usually when oral prednisolone was tapered to around 0·1 mg kg-1 . In mucocutaneous pemphigus vulgaris (mcPV), the initial doses (mean ± SD) of prednisolone were significantly lower in patients with relapse (0·78 ± 0·24 mg kg-1 ) than in those without relapse (1·01 ± 0·01 mg kg-1 ). At relapse, mcPV shifted to mucosal dominant PV (mPV; 40%), pemphigus foliaceus (PF) (20%) or 'other' (20%). In contrast, relapsing mPV and PF had the same clinical phenotypes as the initial phenotypes. Patients with both anti-desmoglein (Dsg)1 and anti-Dsg3 antibodies at onset had recurrence with anti-Dsg3 antibodies alone (40%), with both anti-Dsg1 and anti-Dsg3 antibodies (30%), with anti-Dsg1 antibody alone (20%) or were subthreshold (10%). CONCLUSIONS: mcPV shows transitions in clinical phenotype and autoantibody profile at relapse. At least 1 mg kg-1 daily of prednisolone, especially for patients with mcPV, and prudent tapering around 0·1 mg kg-1 may lead to better outcomes.
