TOXIC EPIDERMAL NECROLYSIS COMPLICATED BY CHRONIC INFLAMMATORY KERATITIS/CONJUNCTIVITIS AND INFLAMMATORY PERIPHERAL NEURITIS

Abstract

Introduction We present a patient who developed chronic keratitis and conjunctivitis, inflammatory neuropathy of the hands, and onychomadesis as complications of her toxic epidermal necrolysis (TEN). Neuropathy was treated with glucocorticoids, methotrexate, and high dose IVIG; however the keratitis and conjunctivitis were resistant and required adalimumab. To the best of our knowledge, there are no other reports of inflammatory neuropathy as a sequelae of Stevens Johnson Syndrome (SJS)/TEN, nor management of chronic ocular inflammation with adalimumab. Case Description A 33-year old woman with stable lupus (manifested mainly with malar rash and arthralgias and maintained on hydroxychloroquine) developed TEN after receiving doxycycline for a c-section incision site infection. As complications of her TEN, she developed onychomadesis, painful inflammatory neuropathy in the fingers and hands, and severe chronic keratitis and conjunctivitis. Her peripheral neuropathy responded to methotrexate, prednisone, and high dose IVIG. However, the chronic keratitis and conjunctivitis failed these therapies, ultimately leading to corneal ulcerations requiring grafts. Treatment with adalimumab resulted in improvement of her vision. Discussion In several case reports, SJS/TEN has been associated with chronic ocular inflammation as a complication, for which glucocorticoids have shown benefit. We have found no other cases of inflammatory peripheral neuropathy as a complication of TEN. We also report some success in the treatment of chronic keratitis and conjunctivitis (as complications of TEN) with adalimumab. Therefore, immunosuppressive biologic agents such as adalimumab may have value in the treatment of chronic ocular inflammatory complications resulting from SJS/TEN.