Dermatosis ampollar IgA lineal: serie de 17 casos

Estudio retrospectivo de 10 pacientes adultos con enfermedad ampollar autoinmune por inmunoglobulina A lineal

“…Ninguno se asoció al uso de vancomicina, el fármaco con mayor frecuencia asociado a EIAL 9 . Las comorbilidades encontradas de artritis reumatoide, lupus eritematoso discoide y alopecia areata coinciden con las halladas en la literatura [2][3][4] . Todos los pacientes tuvieron un compromiso de tres o más áreas anatómicas entre el dorso, el tórax, los glúteos, el abdomen y los miembros como se describe en otras publicaciones (Fotos 2, 6 y 8) [2][3][4] .…”

“…La causa que determina la aparición de la EIAL en diferentes momentos de la vida estaría asociada a la expresión de ciertos genes del complejo mayor de histocompatibilidad como DQ2, B8, DR3 y CW7 4,5 . Se trata de una enfermedad infrecuente cuya incidencia se estima entre 0,5 y 2,3 casos por millón de habitantes por año 6 .…”

Linear Immunoglobulin a Bullous Dermatosis in Children

“…Concerning vaccinations as a LABD trigger in children, two cases have been documented after flu/varicella vaccination (26) and two cases after quadrivalent human papillomavirus vaccine (qHPV), respectively in a 14 years old Italian girl (68) and in a 16-year-old Japanese girl (69). It was assumed that, as in rhesus macaques, the qHPV vaccine induces a Th2-skewed immune response with high titers of IgA and IgG1 antibodies, and a high level of IgA could also occur in humans after qHPV vaccine (69).…”

“…A heterogeneous humoral response characterizes LABD. Indirect immunofluorescence (IIF) with salt-split skin was performed to detect circulating autoantibodies anti-BMZ, and its sensitivity ranges between 30% and 50% (26). However, as reported by Antiga et al (39), circulating IgA are not an exclusive finding in LABD since they could be detected in other subepidermal blistering diseases too (85,86).…”

“…The primary treatment of LABD is initially dapsone 0.5 mg/kg/ day, which is gradually increased until signs resolve and symptoms are controlled (usually up to 2 mg/kg/day) (9, 10), with a quick response in most of the cases (18,24,25,90). It is effective as monotherapy or in combination with corticosteroids, antibiotics, or colchicine (26). Treatment with dapsone can have adverse effects such as hemolysis in glucose-6-phosphate dehydrogenase (G6PD) deficient patients, methemoglobinemia, bone marrow suppression, and peripheral neuropathy.…”