Article information
Resumen
El lupus ampolloso es una manifestación poco frecuente del lupus eritematoso sistémico (LES). Otras enfermedades ampollosas tales como el penfigoide ampolloso, epidermólisis ampollosa adquirida, dermatosis ampollosa Ig A lineal y dermatitis herpetiforme también han sido informadas en LES. Describimos un paciente que desarrolló lesiones ampollosas 14 días luego de iniciar terapia con altas dosis de glucocorticoides y ciclofosfamida para manifestaciones severas del LES (nefritis y hemorragia alveolar). Se confirmó el diagnóstico de lupus ampolloso. La respuesta al tratamiento con dapsona fue notable a las 48 horas. En este artículo revisamos la epidemiología, hallazgos clínicos, histopatológicos e inmunopatológicos; el diagnóstico diferencial y el tratamiento del LES ampolloso.
Palabras clave:
lupus eritematoso sistémico ampolloso
dapsona
lupus cutáneo
Summary
Bullous systemic lupus erythematosus (BSLE) is an unusual finding in systemic lupus erythematosus (SLE). Other bullous disorders such as bullous pemphigoid, epirdermolysis bullosa acquisita, linear IgA bullous dermatosis and dermatitis herpetiformis has also been reported in association with SLE. We described a patient who developed severe bullous lesions 14 days after high-dose systemic glucocorticoids and cyclophosphide therapies were initiated for severe SLE manifestations (nephritis and alveolar hemorrhage). A diagnosis of bullous SLE was made. Therapy with dapsone resulted in a marked clinical improvement of the bullous eruption within 48 hours. This article also discusses the epidemiology, clinical, histopathologic and immunopathologic features, differential diagnosis and the treatment of BSLE.
Key words:
bullous systemic lupus erythematosus
dapsone
cutaneous lupus
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