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

 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. History

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

 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 months Treatment

 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 Follow up – Day 7

Histopathology  limbal region LE  Polymorphonuclear cells in ENL

Ciliary body ENL

POLYMORPHS IN ENL

 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 Treatment Options

Ocular leprosy Bacilli in the eye Type IIType I

Ocular Leprosy Type 1(Reversal reaction)Type 2 (ENL) Immune statusSpontaneous enhancement of Cellular immunity Spontaneous enhancement of Humeral immunity Ocular findings5 th and 7 th nerve palsyEpiscleritis, 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