Eosinophilic fasciitis (Shulman syndrome) is a chronic inflammation primarily of the septums and fascia, and is characterized by the infiltration of eosinophils with additional similarities to systemic sclerosis. Several diseases have been described in association with eosinophilic fasciitis. Among these are aplastic anaemia, haemolytic anaemia, thrombocytopenia, lymphoproliferative disorders, thyroiditis, pulmonary fibrosis, Sjögren's syndrome, Raynaud's phenomenon, myositis, medium vessel vasculitis, pericarditis, colitis and glomerulonephritis. To date, no association with congenital asplenia has been described. We report the case of a woman with eosinophilic fasciitis and congenital asplenia and discuss the possible causes and potential consequences.
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http://dx.doi.org/10.1007/s00393-009-0478-7 | DOI Listing |
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