histoid leprosy by aseem
TRANSCRIPT
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HISTOID LEPROSY
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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
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INTRODUCTION
First described as a subtype of LL
Pts on long-standing DAPSONE Monotherapy
Initial Improvement Relapse
Drug Resistance / Inadequate treatment / Mutant Organisms
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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.
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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 ENLKaur, 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
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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
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CLINICAL FEATURES
CLASSIFICATION OF LESIONS :
Subcutaneous NodulesDeeply fixed Cutaneous NodulesSuperficially placed Cutaneous NodulesSoft NodulesPlaques
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SUBCUTANEOUS NODULE
aa
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CUTANEOUS NODULES
n
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KOEBNERIZATION
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CUTANEOUS PLAQUES
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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
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DIFFERENTIAL DIAGNOSES
Conventional Lepromatous nodules / LLENLVon Recklinghausen’s disease / NF-1Molluscum ContagiosumKeloidsPKDLCutaneous SarcoidosisDermofibromaCutaneous Tumours
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DIAGNOSIS
HISTORY Treatment history (Duration / Drugs /
Compliance)CLINICAL FEATURES
Histoid Facies / Characteristic Papulo-Nodular cutaneous lesions over apparently normal skin
INVESTIGATIONSSSSHPEIMMUNOLOGY
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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)
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a
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INVESTIGATIONS
HPE
Epidermal AtrophyMild AcanthosisFlattening of Rete Ridges
Dermal Histoid Lepromas – Expansive , well –circumscribed granulomas, covered by Pseudocapsule
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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
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TUBERCULOID CONTAMINATION
Subcutaneous Histoid Lepromas
Definite foci of epitheloid cells located withinlesion substance or encircling fibrous capsule
? Tuberculoid component of Earlier Borderline phase
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INVESTIGATIONS
IMMUNOPROFILE (Sehgal et al)
Lowered cell mediated immunityEnhanced humoral immune
response (Raised IgG, IgA, IgM)Diminished complements
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TREATMENT
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