Peripheral ulcerative keratitis - an extracutaneous neutrophilic disorder:Report of a patient with rheumatoid arthritis, pustular vasculitis, pyoderma gangrenosum, and Sweet's syndrome with an excellent response to cyclosporine therapy

The term peripheral ulcerative keratitis represents a spectrum of inflammat ory diseases, characterized by cellular infiltration, corneal thinning, and ulceration. Neutrophilic dermatoses are rarely associated with peripheral ulcerative keratitis. To date, peripheral ulcerative keratitis has only bee n reported in patients with pyoderma gangrenosum. Separate episodes of pyod erma gangrenosum, Sweet's syndrome, and pustular vasculitis developed in a 60-year-old patient with rheumatoid arthritis over an 8-year period. Over t he past 2 years, 3 episodes of peripheral ulcerative keratitis occurred. Cy closporine (4 mg/kg/d) treatment was started on confirmation of pyoderma ga ngrenosum. Over the ensuing 2 years, it became evident that the activity of her ocular and skin diseases, as well as her arthritis, paralleled the adm inistration or cessation of cyclosporine therapy. Dermatologists should be aware of the association of Sweet's syndrome, pyoderma gangrenosum, and pus tular vasculitis with peripheral ulcerative keratitis. This rare ocular man ifestation and the serious sequelae when left untreated make recognition cr ucial. Cyclosporine proved to be a very effective treatment for all of our patient's diseases.