Leprosy Ocular Erythema nodosum leprosum S.R. Rathinam FAMS PhD Uveitis service Aravind Eye Hospital Madurai.

History 45 year old male patient with lepromatous leprosy he was advised treatment several times, but because of recurrent ENL reaction, patient never took proper treatment.

First Examination OS: leproma with hypopyon aqueous tap, staining for Mycobacterium lepra + hypopyon is reported but very rare in leprosy

Treatment Standard multi drug anti leprosy treatment 20 mg oral steroids 600 mg Rifampicin and 300 mg Clofazimine on day 1 of every month 100 mg Dapsone daily and 50 mg Clofazimine for 12-24 months

Follow up - Day 7 developed very severe type 2 reactions, experienced fever, malaise, and polyarthralgia as well as an abrupt onset of numerous tender, erythematous nodules over the face, extremities and over the trunk. This is termed erythema nodosum leprum (ENL) and tends to occur with poor cellular immunity but strong humoral immunity to M. leprae, more specifically in patients with high bacillary load

Follow up – Day 7 severe ocular ENL resulted in ocular perforation because of severe pain the globe was enucleated histopathological staining

Histopathology limbal region LE Polymorphonuclear cells in ENL

Ciliary body ENL

POLYMORPHS IN ENL

Treatment Options Corticosteroids are highly useful for type 1 and type 2 reactions, but Thalidomide is the most effective agent to treat severe forms of type 2 reactions Thalidomide was not available freely but would have helped this patient

Ocular leprosy Bacilli in the eye Type II Type I

Type 1(Reversal reaction) Ocular Leprosy Type 1(Reversal reaction) Type 2 (ENL) Immune status Spontaneous enhancement of Cellular immunity Spontaneous enhancement of Humeral immunity Ocular findings 5th and 7th nerve palsy Episcleritis, Scleritis, Uveitis Uveitis was found in 56% In HPE Clinically 5-6 %

Type III Hypersensitivity Erythema nodosum leprosum (ENL) is induced in a Type 2 lepra reaction Type 2 lepra reactions are acute inflammatory complications that often present as medical emergencies during the chronic course of treated or untreated Hansen's disease