Effect of Cortisone on Rheumatoid Nodules

VAN DER HOEVE (1931), basing his observations on two cases, drew attention to a condition characterized by the spontaneous appearance of scleral perforations, which he named scleromalacia perforans. A somewhat similar case had previously been described by Holthouse (1893), and Kuhnt (1912) reported a case of scleral perforation associated with hydroa vacciniforme. Under the term scleritis necroticans Rochat (1933) described two further cases but he was later willing to accept the earlier designation. Van der Hoeve (1934) reviewed his original material with that of Rochat and referred to two additional cases. He noted that the digease was bilateral in most patients and began in the anterior sclera with yellow or greyish subconjunctival nodules, associated with a gradually developing scleral necrosis, progressing to perforation with exposure of the uvea. Three of these cases presented a history of pre-existing chronic articular disease of an ankylosing type and this feature he associated with the ocular condition to form a distinct syndrome. Following upon van der Hoeve's recognition of this diseaseentity, further cases were reported by Eber(1934), Wojno (1935), Kiehle (1937), Cast (1937), Soriano and Riva (1937), and Urrets Zavalia and others (1937). Verhoeff and King (1938) reviewed fourteen cases which they had at that time been able to trace in the literature and described a fifteenth in which the eye had been enucleated, thus providing the first complete histological report of the condition. They were able to confirm the features of the ocular manifestations as originally described and emphasized the association with rheumatoid arthritis. They were the first to point out that the histological changes in the scleral nodules were essentially similar to those of the subcutaneous nodules of rheumatoid arthritis, and they suggested that the reaction might be due to the deposition of a chemical substance arising in the course of some metabolic disturbance; the deposition of urates in gout was an analogous process. Indeed it is interesting to note that more recently van der Hoeve (1948) has advanced the theory that scleromalacia perforans arises from a disturbance of the lipoid and cholesterol metabolism, and that he associates it with the group of diseases which includes Hand-Schilller-Christian disease, Niemann-Pick disease, Tay-Sachs disease, and xanthomatosis.