The term "collagen diseases" was first used by Klemperer1 to stress the importance of the connective tissue reaction in, and to stimulate investigation of, a group of diseases with heterologous clinical manifestations. The most obvious pathologic feature which is found in each of these diseases is a fibrinoid degeneration of the connective tissue. Neumann,2 in 1880, thought that this type of reaction was due to a transformation of the collagen fibers into fibrin-like material. Recently, however, Altshuler and Angevine3 have concluded that the homogenous ground substance, and not the collagen fiber, is the anatomic site of the connective tissue alteration and that the fibrinoid deposits are a result of a precipitation of mucopolysaccharides by the action of a basic protein. Thus, it appears that the term "systemic diseases of the connective tissue" or "dysgammaglobulinemia" is a more appropriate designation than collagen diseases.4 However, since the term
MAUMENEE AE. Ocular Manifestations of Collagen Diseases. AMA Arch Ophthalmol. 1956;56(4):557–562. doi:10.1001/archopht.1956.00930040565004
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