Progressive Keratolysis with Pseudopterygium Associated with Erythema Elevatum Diutinum

Objective To report a patient with a rare case of progressive keratolysis with pseudopterygium associated with erythema elevatum diutinum (EED). Design Interventional case report and literature review. Participant One patient with ocular presentation of EED was identified. Methods A 64-year-old woman presented with recurrent pterygium and painful corneal thinning with impending perforation of the left eye. Examination revealed intensely inflamed pseudopterygium, paracentral and peripheral corneal ulcerations, and descemetocele with a pinpoint leakage. Main Outcome Measures Clinical course and laboratory and histopathologic findings. Results Disseminated skin lesions developed during hospital admission. Examination revealed multiple, well-demarcated, discrete, red to violaceous, firm nodules and plaques on the knuckles and dorsum of the hands, and the extensor surface of the forearms, elbows, knees, right ankle, and buttocks. A skin biopsy of the recent lesions showed a dense and diffuse inflammatory cell infiltration, predominantly neutrophilic infiltrates and nuclear dust (leukocytoclasis), located around small blood vessels in the dermis, consistent with the diagnosis of EED. The diagnosis of progressive keratolysis with pseudopterygium associated with EED was made. Spontaneous, rapid resolution of both cutaneous and ocular lesions occurred after dapsone therapy. Conclusions Erythema elevatum diutinum is a rare, chronic, and recurrent disease that has both dermatologic and ocular manifestations. Peripheral ulcerative keratitis seems to be the most common ocular finding and may be the initial presenting feature of EED. Ophthalmologists should be aware of this rare entity in the differential diagnosis of ulcerative or nonulcerative peripheral keratitis. Financial Disclosure(s) The author(s) have no proprietary or commercial interest in any materials discussed in this article.