Churg-Strauss syndrome: a disabling disease.

M C André, A Fraga, M Duarte Reis, P Filipe, L M Soares de Almeida, M A Marques Gomes

Abstract


Churg-Strauss syndrome (CSS) is an infrequent vasculitis that affects small to medium-sized vessels. We describe a 51 year-old-female admitted to our inpatient unit with bullae on her right foot and forearm with pain, paresthesias and impotence of the foot. There was rapid clinical deterioration with lost of gait and peripheral eosinophilia. Histopathology showed many extravascular eosinophils. Bone marrow had an increased number of eosinophils and their precursors with no neoplastic cells infiltration. Electromyogram revealed mononeuritis multiplex with bilateral sciatic and right femoral nerve involvement. She fulfilled the eligibility criteria of American College of Rheumatology (ACR) and Chapell Hill Conference Consensus (CHCC) of CSS so corticosteroids and cyclophosphamide and rehabilitation program were begun with good clinical and laboratorial response. This report illustrates the importance of identifying atypical cutaneous features of CSS for the early diagnosis of this rare condition and the role of a multidisciplinary team in this multissystemic disease.

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