Typical and atypical manifestations of leprosy

Typical and atypical manifestations of leprosy

1. Typical And Atypical Manifestation Of Leprosy Dr ASHWINI N 2 ND YEAR POSTGRADUATE DEPT OF DERMATOLOGY KIMS HUBLI

2. Definition Etiology Clinical manifestation Laboratory diagnosis Treatment

3. Leprosy is a chronic disease caused by Mycobacterium leprae , infectious in some cases, and affecting the peripheral nervous system, the skin, and certain other tissues. Definition

4. “ There is hardly anything on earth, or between it and heaven which has not been regarded as the cause of leprosy; and this is but natural, since the less one knows, the more actively does his imagination works”. Gerhard Armauer Hansen demonstrated Lepra bacilli in the year 1873. Etiology

5. Rod shaped bacterium, 1-8micron in length and 0.3 micron in diameter, with parallel sides and round borders. Acid fast bacilli Pyridine extraction test differentiate from other mycobacterium. Cannot be grown in artificial medium, but can be grown in mouse foot pad and nine banded armadillo Mycobacterium leprae

6. A case of leprosy is defined as the individual who has not completed the course of treatment and has one or more of the three cardinal signs Hypopigmented or erythematous skin lesion(s) with definite loss/impairment of sensation Involvement of the peripheral nerves, as demonstrated by definite nerve thickening with sensory impairment Skin smear positive for AFB WHO Case definition

7. • Madrid Classification • Indian Classification • Ridley- Jopling Classification • World Health Organization Classification (1988) For Leprosy Control Programs Classification

8. • Tuberculoid leprosy (TT) • Borderline tuberculoid leprosy (BT) • Mid-borderline leprosy (BB) • Borderline lepromatous leprosy (BL) • Lepromatous leprosy (LL) Ridley- Jopling classification (1966)

9. Clinical features

10. Indeterminate Leprosy Initial lesion in 80% of patient Ill defined patch Face, trunk and thighs Sensation variable Histopathology shows perivascular and adnexal granuloma with or without few bacilli

11. Tuberculoid Leprosy Single or few,asymmetrical , well defined erythematous or copper colored patches Sensation is absent Nerves- thickened, presence of feeding nerves, abscess Skin smear-negative Lepromin test-strongly positive Course-relatively benign and stable, with good prognosis

12. Borderline Tuberculoid Leprosy Few assymetrical hypopigmented or skin colored macules , plaque with ill defined margins Presence of satellite lesions near the advancing margin of patch Sensory impairment is marked Nerve involvement marked and assymentrical

13. Mid Borderline Leprosy

14. Unstable form, reaction is frequent Annular lesions with characteristic punched out appearance( inverted saucer shaped Sensory impairment moderate Nerve involvement marked and asymmetrical

15. Borderline Lepromatous Leprosy Multiple shiny macules , papules, nodules and plaques with sloping edges Sensory impairment slight Nerve involvment widespread and less asymmetical Glove and stocking hyoasthesia

16. Lepromatous Leprosy

17. Hypopigmented , erythematous or coppery, shiny macules , papules, nodules Lesions symmetrically distributed, small, multiple, shiny with normal or mild sensory loss Leonine facies - infiltration of the skin , loss of eyebrows and eyelashes Nerve- symmetrical glove and stocking anaesthesia Lepromin test negative

18. Histiod Leprosy Occurs as a consequence of dapsone monotherapy or inadequate therapy Appear as smooth, shiny, hemispherical, dome-shaped, nontender soft to firm nodules Usually located on the face, back, buttocks and extremities and over bony prominences,especially around the elbows and knees.

19. Lucio Leprosy ( lepra bonita , beautiful leprosy) Diffuse form of LL, commonly seen in mexico Mycobacterium lepromatosis , was isolated Slowly progressive diffuse Infiltration of skin of face and most of the body Loss of body hair, loss of eyebrows & eyelashes, and widespread sensory loss

20. Slit skin smear Histopathalogical examination Nerve conduction studies Nerve biopsy I nvestigation

21. Three types: 1.Type 1 reactions associated with cell-mediated hypersensitivity. 2.Type 2 reactions associated with immune complexes. 3.Lucio phenomenon, associated with necrosis of arterioles whose endothelium is massively invaded by M.leprae . Lepra Reaction

22. Associated with a rapid change in cell-mediated immunity(CMI) following treatment. Typically seen in borderline leprosy. Type 1 reactions

23. Pre-existing skin patches or plaques become erythematous , swollen and may be tender looking like erysipelas. Crops of fresh inflamed skin lesions in the form of plaques may appear Severe pain\tenderness of one or more peripheral nerves Clinical features

24. TUBERCULOID LEPROSY IN TYPE 1 LEPRA REACTION

25. An immune complex syndrome (antigen-antibody reaction involving complement). Type III hypersensitivity reaction (Coombs and Gell ). Occurs almost exclusively in lepromatous leprosy ( LLp and LLs), occasionally in BL. TYPE 2 REACTIONS

26. No clinical change is noticed in the original clinical skin lesions ENL lesions: there is sudden appearance of crops of evanescent (lasting for few days) pink (rose) colored tender papules, nodules or plaques of variable size. ENL lesion when becomes vesicular, pustular , bullous and breakdown to produce ulceration called as erythema nodosum necroticans Associated with systemic symptoms like fever joint pain Neuritis Clinical feature

27. Corticosteroids are the treatment of choice- for neuritis & ulcerations . Dose of prednisone If symptoms are not controlled within 24-48 hours, dose increased in 20-40mg/day increments. Attempt to taper off over 2-3 months. Treatment of Type 1 reactions

28. Systemic steroids form the main stay of treatment. Prednisolone started as 1mg/kg and gradually tapered over 2-3 months Thalidomide – also effective in the control of reaction. Started in a dose of 100mg 3-4 times daily, Will usually control the reaction within 48 hours. Tapered off after reaction controlled. Management of type 2 reactions

29. 27-year-old man Red-brown plaques and nodules over extremities and face of 3 year duration Thick, dry skin over the soles F/s/o lepromatous leprosy was present like madarosis , infitration of face and ulcer over insensitive hands Biopsy of the sole showed lymphocytic infiltrate and numerous acid-fast bacilli seen on fite’s stain Improved on multidrug treatment Case report 1

30. 44-year-old female c/o tingling and numbness of hands and feet dark patches over the face, trunk and extremities of 20 days duration O/E revealed multiple, large, ill-defined hyperpigmented patches over the trunk, extremities, palms and soles Bilateral ulnar , radial cutaneous and lateral popliteal nerves were enlarged and nontender . A clinical diagnosis of BT leprosy was made CASE REPORT 2

31. Well-defined granulomas showing epithelioid cells, langhans giant cells and lymphocytes were seen in the dermis

32. A 50-year-old woman h/o episodes of painful, raised lesions over the body of 15 days duration associated with constitutional symptoms O/E urticarial plaques studded with pseudovesicles upper back and the extensor aspects of the upper limbs HPE f/s/o ENL CASE REPORT 3

33. Leprosy is a chronic inflammatory disease with varied clinical features Early diagnosis and prompt treatment prevents deformity Patient education about the leprosy reaction is important for follow up and compliance to drug Histopathological examination plays a important role in case of unusual presentation Conclusion

34. Thank you