Eosinophilic fasciitis and aplastic anaemia

A 51-year-old female presented in 1984 with severe bilateral conjuctival oedema which failed to respond to corticosteroid or adrenaline eye drops. The pathogenesis of AA associated with EF remains uncertain. The association of EF with autoimmune disorders such as immune thrombocytopenia, pernicious anaemia, Grave's disease and thyroiditis (Schulman et al. 1979; Mazanec 1982; Smiley, Hussain & Indenbaum 1982), and the demonstration of a humoral inihibitor of haemopoiesis, probably an autoantibody, in the serum of a patient with AA and diffuse fasciitis (Hoffman et al. 1979) suggest that the AA may be immune mediated in some patients. Other possible mechanisms for the AA include abnormalities of marrow microenvironment, eitehr an abnormality of bone marrow collagen or abnormal proteoglycan, and cellular cytotoxicity by an undefined agent affecting both haemopoietic stem cells and fibroblasts (Hoffman 1984). The pathogenesis of the fascial proliferation in EF also remains unknown. Immune mechanisms have been suggested based on the detection of elevated concentrations of circulating immune complexes (Seibold et al. 1982) and the demonstration of immunoglobulin deposits in the subcutis (Barnes et al. 1979). This may attract eosinophils which in turn stimulate the tissue fibrosis (Hoffman 1984). However, normal levels of complement components in most patients, absence of autoantibodies and inconsistency of immunofluorescent findings do not support this hypothesis.