Modern treatment of severe "shrinkage of the conjunctiva".

COOPER (1858) first described the ocular manifestations of pemphigus. von Kries (1878), working in von Graefe's clinic, described "essential shrinkage of the conjunctiva", and von Graefe (1879) identified the terminal state of pemphigus with essential shrinkage of the conjunctiva. By 1892, when Duane translated Fuchs's textbook into English, the diagnosis of pemphigus conjunctivae was established and three clinical pictures were described: (1) Lesions of the conjunctiva associated with "pemphigus vulgaris of the skin"; (2) Lesions of the conjunctiva with lesions of the mucous membrane, the skin being healthy; (3) Conjunctival lesions without lesions elsewhere. It is now known that the lesions described are not associated with pemphigus vulgaris or any other variety of true pemphigus, but it was not until 1919 that Thost made a plea that "benign mucous membrane pemphigus" be distinguished from other forms of pemphigus. That this was in fact a different disease was established by the later histo-pathological studies of Civatte (1949), confirmed by Lever (1951), which showed that the bullae of benign mucous membrane or ocular pemphigus were subepidermal, whereas those of true pemphigus were intra-epidermal, characterized by acantholysis. In addition, the prognosis for life is vastly different in the two conditions. Rycroft (1934) further elucidated the picture with a survey of the literature and an account of five cases; these he divided into: -(i) acute forms-occurring between 2 and 35 years. (ii) chronic forms-occurrng between 40 and 70 years. That the acute forms are almost entirely cases of erythema multiforme exudativum was advanced by Klauder and Cowan (1942) and by Lever and Talbott (1942). This disease was described by Hebra (1866), but has become commonly known as Stevens-Johnson syndrome since the account of two cases with severe ocular manifestations by Stevens and Johnson (1922). In addition to these two conditions, the picture of sub-conjunctival shrinkage can be produced by drug sensitivity, and one such case is included in this paper. Hansen (1950), in describing eight cases, suggested that hypersensitivity to drugs should be suspected in all cases.