Histoid leprosy by aseem

1. HISTOID LEPROSY

2. HISTORY Coined by Wade in 1960 Gk : ‘ Histo ’ – Tissue ; ‘ Eioid ’ – Resemblance Wade documented MB cases on Dapsone / De Novo Immunological Profiling by Sehgal et al

3. INTRODUCTION First described as a subtype of LL Pts on long-standing DAPSONE Monotherapy Initial Improvement  Relapse Drug Resistance / Inadequate treatment / Mutant Organisms

4. definition Histoid Leprosy is an expression of MB Leprosy, characterized by well-demarcated, Papulo -Nodular cutaneous and subcutaneous lesions over apparently normal skin, with infrequent Reactional episodes.

5. EPIDEMIOLOGY Age distribution : 10 – 84 Y (commonly 20-39 Y) Incidence : 3.2% Sehgal & Srivastava 2.8% Kalla et al Male : Female – 5.7 : 1 ; 20% had ENL Kaur , I., Dogra , S., De, D. and Saikia , U.N. (2009), Histoid leprosy: a retrospective study of 40 cases from India. British Journal of Dermatology, 160: 305–310. doi : 10.1111/j.1365- 2133.2008.08899

6. CLINICAL FEATURES Disseminated, 03-50 in number, Papulonodular , firm  soft, erythematous  Copper Coloured , hemispherical / dome-shaped / umblicated , Shiny glistening lesions over apparently normal underlying skin over Face, Back, Buttocks and Limbs Koebnerization ? Pseudo-isomorphic

7. CLINICAL FEATURES CLASSIFICATION OF LESIONS : Subcutaneous Nodules Deeply fixed Cutaneous Nodules Superficially placed Cutaneous Nodules Soft Nodules Plaques

8. Subcutaneous nodule aa

9. CUTANEOUS NODULES n

10. Koebnerization

11. CUTANEOUS PLAQUES

12. CLINICAL FEATURES FACIES: - Old wrinkled atrophic skin with absent eyebrows - Normal facies without any manifestation of leprosy Eyebrows and nasal mucosa (cartilage ) are spared in most cases

13. Differential diagnoses Conventional Lepromatous nodules / LL ENL Von Recklinghausen’s disease / NF-1 Molluscum Contagiosum Keloids PKDL Cutaneous Sarcoidosis Dermofibroma Cutaneous Tumours

14. DIAGNOSIS HISTORY Treatment history (Duration / Drugs / Compliance) CLINICAL FEATURES Histoid Facies / Characteristic Papulo - Nodular cutaneous lesions over apparently normal skin INVESTIGATIONS SSS HPE IMMUNOLOGY

15. INVESTIGATIONS SSS Abundant Uniform Solid-Stained Red long rods with tapering ends Bacilli / few Globi (lack of Globea , matrix substance secreted by metabolism of bacilli)

16. a

17. INVESTIGATIONS HPE Epidermal Atrophy Mild Acanthosis Flattening of Rete Ridges Dermal Histoid Lepromas – Expansive , well –circumscribed granulomas, covered by Pseudocapsule

18. hpe Layers of cells in Granuloma Innermost layer - Polygonal Periphery - Spindle shaped histiocytes , containing long AFB arranged in groups along long axis – ‘HISTOID HABITUS’ Numerous Solid-stained bacilli, few Globi Absence of ETS / Foam (ETS hampers Bacillus metabolism and death)  preserved Bacilli in lepra (Virchow) cells

19. TUBERCULOID CONTAMINATION Subcutaneous Histoid L epromas Definite foci of epitheloid cells located within lesion substance or encircling fibrous capsule ? Tuberculoid component of Earlier Borderline phase

20. investigations IMMUNOPROFILE ( Sehgal et al) Lowered cell mediated immunity Enhanced humoral immune response (Raised IgG , IgA, IgM ) Diminished complements

21. TREATMENT

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