Effect of Cortisone on Rheumatoid Nodules of the Sclera (Scleromalacia Perforans)

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 disease- entity, further cases were reported by Eber(1934), Wojno (1935), Kiehle (1937), Cast (1937), Soriano and Riva (1937), and Urrets Zavalia and others (1937).