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Hansen’s Disease. Ma. Bernadette V. Lopez-Dee Adrian B. Lorenzo. Epidemiology . Global prevalence – 1.25 cases per 10,000 persons India – 80% worldwide Male to female ratio of occurrence – 1.5 : 1 Age of occurence – before 35 (endemic areas), age 50 (exposure to armadillos)
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Hansen’s Disease Ma. Bernadette V. Lopez-Dee Adrian B. Lorenzo
Epidemiology • Global prevalence – 1.25 cases per 10,000 persons • India – 80% worldwide • Male to female ratio of occurrence – 1.5 : 1 • Age of occurence – before 35 (endemic areas), age 50 (exposure to armadillos) • Latency period – 5 years (paucibacillary), 20 years (multibacillary) • Mode of transmission – armadillo exposure, other cases of Hansen’s disease Close contact – 28 % (household contacts) - 80 % (neighbors, social contacts)
Infectious agent Mycobacterium leprae • Weakly acid-fast organism • Grows best at 30 ̊C below human body core temperature • Long generation time (12-14 days) • Unable to be cultured in vitro
Diagnosis • Identification of infectious organism in affected tissue • Skin biopsies: from skin or nerve lesions – stained for bacillus with Fite-Faraco stain • Slit smears: from lesions and cooler areas of skin – stained with Acid-fast stain • organisms found – multibacillary • 5 or few lesions/ negative – paucibacillary
Classification Early and Indeterminate leprosy Tuberculoid leprosy Borderline tuberculoid leprosy Borderline leprosy Borderline lepromatous leprosy Lepromatous leprosy Histoid leprosy
1. Early and Indeterminate Leprosy • Insidious onset • Numbness – first manifestation in 90% of patients • Loss of senses of cold and light touch - earliest sensory changes • Solitary, ill-defined hypopigmentedmacule- often the first lesion • Erythematous macules (cheeks, upper arms, buttocks, thighs) –less often
1. Early and Indeterminate Leprosy • Biopsy – usually no bacilli or only a few • Few cases remain in this state • Evolve to lepromatous, tuberculoid, or borderline types • Good cell-mediated immunity – often resolve spontaneously or never develop other signs and symptoms of Leprosy
2. Tuberculoid Leprosy • Solitary lesions or few in number (5 or less) • Asymmetrical distribution • Lesions – hypopigmented or erythematous, dry, scaly, hairless • Typical lesion – large, erythematous plaque w/ a sharply defined & elevated border that slopes down to a flattened atrophic center – “a saucer right side up” • Common locations – face, limbs, or trunk • Lesion is anesthetic & anhidrotic
2. Tuberculoid Leprosy • Nerve involvement – early & prominent • Greater auricular nerve & Superficial peroneal nerve – visibly enlarged • Atrophy of interosseous muscles of hand, w/ wasting of thenar & hypothenar eminences, contracture of fingers, facial muscle paralysis, foot drop • Evolution generally slow • Often, there is spontaneous remission in 3 years, or less if w/ treatment
3. Borderline Tuberculoid Leprosy • Similar to tuberculoid lesions, but smaller and more numerous • Characteristic – satellite lesions around large macules or plaques
4. Borderline Leprosy Lesions: • Numerous but countable, consist of red, irregularly shaped plaques • Small satellite lesions may surround larger plaques • Generalized but asymmetrical • Edges are not so well defined • Nerves may be thickened and tender • Anesthesia is only moderate
5. Borderline Lepromatous Leprosy • Lesions are symmetrical, numerous (not countable), may include macules, papules, plaques, and nodules • Small lepromatous lesions > larger borderline type lesions • Nerve involvement -appears later, symmetrical • Nerves - enlarged and/or tender • Sensation & sweating over individual lesions are normal • Usually no features of full-blown leprosy (madarosis, keratitis, nasal ulceration, leonine facies)
5. Borderline Lepromatous Leprosy Hyperpigmentation due to Clofazamine
6. Lepromatous Leprosy • Cutaneous lesions – pale lepromatousmacules / lepromatous infiltrations w/ numerous bacilli • Becomes progressively worse w/o treatment • Divided into polar form LLp & subpolar form LLs • Macular lepromatous lesions- diffuse & symmetrical distribution, small, numerous, ill-defined, blend into surrounding skin • No loss of sensation over lesions, nerve thickening, nor changes in sweating • Slow, progressive hair loss – outer third of eyebrows, eyelashes, then body. Scalp hair remains unchanged.
6. Lepromatous Leprosy • Lepromatous infiltrations divide into diffuse, plaque, and nodular types Diffuse type • Diffuse infiltration of face (forehead), madarosis, waxy shiny appearance of skin – “varnished appearance” • Diffuse leprosy of Lucio– 1/3 of lepromatous cases. Diffuse infiltration of skin. Localized lepromas do not form. Unique complication – Lucios’s phenomenon (erythemanecroticans)
6. Lepromatous Leprosy • Infiltrations- manifested by development of nodules called lepromas • Early nodules – ill defined, occurs in acral parts (ears, brows, nose, chin, elbows, hands, buttocks, knees) • Nerve involvement – variable, very slow Nerve disease – bilaterally symmetrical, stocking-glove pattern (misdiagnosed as diabetic neuropathy)
6. Lepromatous Leprosy Multiple papules and nodules Enlargement of the earlobe
7. Histoid Leprosy • Uncommon form of multibacillary leprosy • Skin lesions – yellow-red, shiny, large papules and nodules in dermis or subcutaneous tissue • 1- 15 mm in diameter • Appear anywhere, but favor the buttocks, lower back, face, and bony prominences • This pattern may appear de novo, but mostly described in patients w/ resistance to long-acting dapsone resistance