RESUMO
Pyoderma gangrenosum (PG) is a painful, ulcerating neutrophilic dermatosis commonly associated with a variety of underlying systemic conditions. We report a child with neurofibromatosis-1 (NF-1) and an aseptic splenic abscess who developed multifocal PG in areas of iatrogenic skin trauma. There is no clinical evidence or theoretical basis to suggest a causal relationship between NF-1 and PG. Systemic corticosteroid and cyclosporine therapy led to complete resolution of the lesions.
Assuntos
Abscesso/etiologia , Neurofibromatose 1/complicações , Pioderma Gangrenoso/etiologia , Esplenopatias/etiologia , Abscesso/tratamento farmacológico , Criança , Clobetasol/uso terapêutico , Quimioterapia Combinada , Glucocorticoides/uso terapêutico , Humanos , Imunossupressores/uso terapêutico , Masculino , Pioderma Gangrenoso/tratamento farmacológico , Esplenopatias/tratamento farmacológico , Tacrolimo/uso terapêuticoRESUMO
We present a patient with Linear IgA who was both difficult to diagnose and treat. Numerous biopsies were needed before a positive result could be returned. Trials of multiple systemic agents failed. Eventually, a second trial of dapsone was successful and well-tolerated. The case exemplifies the determination and perseverance that is often required by both patient and physician in pursuit of symptom relief.