Vasculite crónica fibrosante
- Etiology unknown probably immune complex mediated
- Associated with IBD, Group A Strep, RA, SLE, celiac disease, HIV
- May herald lymphoproliferative disease
- May be associated with an IgA monoclonal gammopathy
- Mecanismo
- Chronic fibrosing leukocytoclastic vasculitis
- Por isso leva a nódulos persistentes eritematosas, violáceos e/ou acastanhados em vez de purpura palpável
- Alguns autores incluem nas dermatoses neutrofílicas
- Apresentação clínica
- Normalmente nas superfícies extensoras
- Rare, chronic disease characterized by symmetric red-violet to red-brown papules and plaques
- Persistent lesions typically over joints
- Histologia
- Lesões precoces
- Vasculite leucocitoclásica
- Neutrophilic infiltrate in upper and mid dermis, involving adnexal structures, with eosinophils
- Lesões mais evoluídas
- Infiltrado menos proeminente
- Já com mais alterações de fibrose
- Abordagem
- Evaluation for associated infection (eg. streptococcal, viral hepatitis, HIV, syphilis), monoclonal gamopathy (serum immunofixation, electrophoresis) or autoimmune disorder
- Tretament
- Drug of choice: dapsone
- Treat underlying infection or systemic disorder
- NSAIDs, niacinamide, tetracyclines, chloroquine, colchicine, plasmapheresis
- Intralesional corticosteroids
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