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Histopathology

  1. Tuberculoid Leprosy (TT)
    A compact granuloma consisting of Langhans giant cells surrounded by well formed rim of lymphocytes with periappendageal and perineural location. The nerves are destroyed beyond recoginition. Ceseation is absent in dermal lesion. AFB cannot be ordinarily demonstrated.
  2. Borderline Tuberculoid Leprosy (BT)
    Epitheloid granuloma with many giant cells and fewer lymphocytes than polar tuberculoid lesion.
  3. Borderline leprosy (BB)
    It is marked by diffuse collections of epitheloid cells, not sorrouded by a rim of lymphocytes and often associated with oedema. Small number of lymphocytes may be seen distributed among the epitheloid cells.
  4. Borderline Lepromatous Leprosy (BL)
    1. Lymphocytes are more in number in comparison to LL type suggesting a better host immune response. Dermal nerves show lympocytic infiltration in addition to presence of M. leprae in Schwann cells and foamy macrophages.
    2.  Sub epidermal clear zone (Grenz zone) is preserved. Proliferation of perineural cells produces a characteristic "cut onion" or "onion peel" appearance.
  5. Lepromatous Leprosy (LL)
    1. Lesion shows sheets of foamy macrophages extensively infiltrating the dermis except for a narrow subepidermidal zone (Grenz zone) which is free of infiltration. Acid fast stain shows macrophages full of M. leprae.
    2. II. Similar collections of macrophages are found in dermal blood vessels, sweat glands, hair follicles and nerves.
    3. Occasional plasma cells may be seen.
    4. Nerves are invariably involved in lepromatous leprosy. Very few lymphocytes are present in skin lesion, indicating a failure of cell mediated immunity.
  6. Histoid Leprosy
    Nodules are characterized by interlacing bundles of spindle shaped histiocytes which may mimic a benign fibrous tissue tumor in H&E. stain. Cells are teeming with M. leprae. Electron microscopy suggests that these cells are modified macrophages.
  7. Diagnosis Leprosy most commonly presents with both characteristic skin lesions and skin histopathology.
    1. Thus the disease should be sus­pected when a patient from an endemic area has suggestive skin lesions or peripheral neuropathy; the diagnosis should be confirmed by his­topathology.
    2. In tuberculoid leprosy, lesional areas-preferably the ad­vancing edge-must be biopsied because normal-appearing skin does not have pathologic features.
    3. In lepromatous leprosy, nodules, plaques, and indurated areas are optimal biopsy sites, but biopsies of normal appearing skin are also generally diagnostic. Lepromatous leprosy is associated with diffuse hyperglobulinemia, which may result in false positive serologic tests (e.g., VDRL, RA, ANA) and therefore may cause diagnostic confusion.
    4. On occasion, tuberculoid lesions may not (1) appear typical, (2) be hypesthetic, and (3) contain granulomas but only nonspecific lymphocytic infiltrates. In such instances, two of these three characteristics are considered sufficient for a diagnosis IgM antibodies to PGL-lare found in 95% of untreated leproma­tous leprosy patients; the titer decreases with effective therapy.
    5. In tuberculoid leprosy-the form of disease most often associated with diagnostic uncertainty owing to the absence or paucity of AFB-patients have significant antibodies to PGL-l only 60% of the time; moreover, in endemic locales, exposed individuals without clinical leprosy may harbor antibodies to PGL-l. Thus PGL-l serology is of little diagnostic utility in tuberculoid leprosy.
    6. Heat-killed M. le­prae (lepromin) has been used as a skin test reagent. It generally elicits a reaction in tuberculoid leprosy patients, may do so in individuals without leprosy, and gives negative results in lepromatous leprosy pa­tients; consequently, it is likewise of little diagnostic value.
    7. PCR of the skin for M. leprae, although positive in LL and BL leprosy, yields negative results in 50% of tuberculoid leprosy cases, again offering little diagnostic assistance.
    8. In lepromatous leprosy, sputum specimens may be loaded with AFB-a finding that can be inappropriately interpreted as representing pulmonary tuberculosis.
  8. Treatment (Medical)​
Multi-drug therapy for Multi-bacillary (MB) leprosy
 

 

RFP

Dapsone

CLF

Adult
50-70kg

600mg/m

100mg/d

50mg/d &
300mg/m

Child
10-14 years(10-14) *

450mg/m

50mg/d

50mg/d &
150mg/m

Less than 10(6-9) *

300mg/m

25mg/d

50mg twice/w &
100mg/m


A compendium of issues and trends in leprosy management: Multi-drug therapy for pauci-bacillary (PB) leprosy

 

 

RFP

Dapsone

Adult
50-70kg

600mg/m

100mg/d

Child
10-14 years(0-5) *

450mg/m

50mg/d

Less than 10(6-14) *

300mg/m

25mg/d


Multi-drug Therapy for Single Lesion Pauci-bacillary (SLPB) Leprosy (ROM)

 

Not recommended for pregnant women and children less than 5 years old
 

 

RFP

OFLX

MNO

Adult weighing   50-70kg
 

600mg

400mg

100mg

Child weighing 5-14 years+
 

300mg

200mg

50mg

  1. Accompanied-MDT(A-MDT)- Give the full course on the first visit
    Uniform MDT (U–MDT)- 6 month to all pts
    1. Management of Reactions:
      NSAIDS (e.g. Ibubrufen), Steroids (e.g. Prednisolone 1-2 mg/kg wt),Chloroquine (e.g. chloroquine phosphate 250mg B.I.D.), Thalidomide, Clofazamine, Antimony compounds, Colchicine, Levamisole, Cyclospoin-A,
    2. Treatment (Surgical)
      Treatment for claw deformity
      Care of trophic ulcers.
      Treatment for foot drop, tarsal disorganisation. Specific deformities of face e.g. nasal deformity. Decompression nerve trunks. Treatment of nerve abscesses.
    3. Reactional States Lepra reactions comprise several common immunologically mediated inflammatory states that cause considerable mor­bidity. Some of these reactions precede diagnosis and the institution of effective antimicrobial therapy.
    4. Type 1 Lepra Reactions (Downgrading And Reversal Reactions)
      1. These reac­tions occur in almost half of patients with borderline forms of leprosy but not in patients with polar disease.
      2. Manifestations include classic signs of inflammation within previously involved macu1es, papules, and plaques and, on occasion, the appearance of new skin lesions, neuritis, and (less commonly) fever-genen~lly low-grade.
      3. The nerve trunk most commonly involved in this process is the ulnar nerve at the elbow, which may be painful and exquisitely tender. If patients with affected nerves are not treated promptly with glucocorticoids irreversible nerve damage may result in as little as 24 h.
      4. The most dramatic manifestation is footdrop, which occurs when the per­oneal nerve is involved.
      5. When type I lepra reactions precede the initiation of appropriate antimicrobial therapy, they are termed downgrading reactions, and the case becomes histologically more lepromatous; when they occur after the initiation of therapy, they are termed reversal reactions, and the case becomes more tuberculoid.
      6. Edema is the most characteristic microscopic feature of type 1 lepra lesions, whose diagnosis is primarily clinical. Reversal reactions are typified by a THI cytokine profile, with an influx of CD4 + helper cells and increased levels of IFNXand IL-2. In addition, type I reactions are associated with large numbers of T cells bearing ϒ/δ receptors­lunique feature of leprosy.
    5. Type 2 Lepra Reactions (Erythema Nodosum Leprot/Cum, Enl)
      1. ENL occurs exclusively in patients near the lepromatous end of the leprosy spectrum (BL-LL), affecting nearly 50% of this group.
      2. Although ENL may precede leprosy diagnosis and initiation of therapy-sometimes, in fact, prompting the diagnosis-in 90% of cases it follows the insti­tution of chemotherapy, generally within 2 years.
      3. The most common leatures of ENL are crops of painful erythematous papules that resolve spontaneously in a few days to a week but may recur; malaise; and lever that can be profound. However, patients may also experience Ijmptoms of neuritis, lymphadenitis, uveitis, orchitis, and glomeru­looephritis and may develop anemia, leukocytosis, and abnormal liver lfunction tests, particularly increased aminotransferase levels. Individ­ual patients may have either a single bout of ENL or chronic recurrent manifestations.
      4. Bouts may be either mild or severe and generalized; mrare instances, ENL results in death.
      5. Skin biopsy of ENL papules reveals vasculitis or panniculitis, sometimes with many lymphocytes but characteristically with poly­morphonuclear leukocytes as well.
      6. Elevated levels of circulating tumor necrosis factor (TNF) have ittll demonstrated in ENL; thus, TNF may playa central role in the pathobiology of this syndrome. ENL is thought to be a consequence of immune complex deposition, given its T H2 cytokine profile and its mgh levels of IL-6 and IL-8.
  2. Lucio’s Phenomenon
    1. This unusual reaction is seen exclusively in patients from the Caribbean and Mexico who have the diffuse form of lepromatous leprosy (lucio leprosy).
    2. Patients with this reaction develop recurrent crops of large, sharply marginated, ulcerative lesions-particularly on the lower extremities-that may be generalized and, when so, are frequently fatal as a result of secondary infection and consequent septic bacteremia.
    3. Histologically, the lesions are characterized by ischemic necrosis of the epidermis and superficial dermis, heavy parasitism of endothelial cells with AFB, and endothelial proliferation and thrombus formation in the larger vessels of the deeper dermis. Like ENL, the Lucio phe­ nomenon is probably mediated by the immune complex.    
  3. Therapy For Reactions
    1. TYPE 1 Type I lepra reactions are best treated with glucocorticoids
    2. Type 2 Treatment of ENL must be individualized.
      1. If ENL is mild (i.e., without fever or other organ involvement, with occasional crops of only few skin papules), it may be treated with antipyretics alone.
      2. n cases with many skin lesions, fever, malaise, and other tissue involvement, brief courses (1 to 2 weeks) of glucocorticoids (initially 40 to 60 mg/d) are often effective. With or without therapy, individual inflamed papules last for> 1 week. Successful therapy is defined by the cessation of skin lesion development and the disap­parance of other systemic signs and symptoms. If, despite two courses of glucocorticoid therapy, ENL appears to be recurring and persisting, treatment with thalidomide (100 to 300 mg nightly) should be initiated, with the dose depending on the initial severity of the reaction.. Clofazimine in high doses (300 mg nightly) has some efficacy against ENL, but its use permits only a modest reduction of the glu­cocorticoid dose necessary for ENL control.
    3. Lucio's phenomenon Neither glucocorticoids nor thalidomide is effec­tive against this syndrome. Optimal wound care and therapy for bac­teremia are indicated. Ulcers tend to be chronic and heal poorly. In severe cases, exchange transfusion may prove useful
  4. Complications
    1. The Extremities Complications of the extremities in leprosy patients are primarily a consequence of neuropathy leading to insensitivity and myopathy. Insensitivity affects fine touch, pain, and heat receptors but generally spares position and vibration appreciation.
      1. Most commonly affected nerve trunk is the ulnar nerve at the elbow, whose involvement results in clawing of the fourth and fifth lingers, loss of dorsal interosseous musculature in the affected hand, and loss of sensation in these distributions.
      2. Median nerve involvement impairs thumb opposition and grasp, while radial nerve dysfunction, though rare in leprosy, leads to wristdrop.
      3. Tendon trans­fers can restore hand function but should not be performed until 6 months after the initiation of antimicrobial therapy and the conclusion episodes of acute neuritis.  
      4. Plantar ulceration, particularly at the metatarsal heads, is probably the most frequent complication of leprous neuropathy.Therapy require careful debridement; administration of appropriate antibiotics; avoidance of weight-bearing until ulcerations are healed, with slowly progressive ambulation thereafter; and wearing of specialized shoes to prevent recurrence.
      5. Footdrop as a result of peroneal nerve palsy should be treated with a simple nonmetallic brace within the shoe or surgical correction atta­ined by tendon transfers. Although uncommon, Charcot's joints, particularly of the foot and ankle, may result from leprosy.
      6. The loss of distal digits in leprosy is a consequence of insensitivity, trauma, secondary infection, and-in lepromatous patients-a poorly understood and sometimes profound osteolytic process. Conscientious protection of the extremities during cooking and work and the early institution of therapy have substantially reduced the frequency and severity of distal digit loss in recent times.
    2. The Nose
      1. In lepromatous leprosy, bacillary invasion of the nasal mu­cosa can result in chronic nasal congestion and epistaxis. Saline nose drops may relieve these symptoms.
      2. Long-untreated LL leprosy may further result in destruction of the nasal cartilage, with consequent saddle-nose deformity or anosmia (more common in the preantibiotic era than at present). Nasal reconstructive procedures can ameliorate significant cosmetic defects.
    3. The EYE Owing to facial nerve palsies, lagophthalmus and corneal insensitivity may complicate leprosy, resulting in trauma, secondary infection, and (without treatment) corneal ulcerations and opacities. For patients with these conditions, eyedrops during the day and oint­ments at night provide some protection from such consequences.
    4. Fur­thermore, in LL leprosy, the anterior chamber of the eye is invaded by bacilli, and ENL may result in uveitis, with consequent cataracts and glaucoma. Thus leprosy is a major cause of blindness in the de­veloping world.
    5. Slit-lamp evaluation of LL patients often reveals "cor­neal beading," representing globi of M. leprae.
    6. The Testes M. leprae invades the testes, while ENL may cause orchitis. Thus males with lepromatous leprosy often manifest mild to severe testicular dysfunction, with an elevation of luteinizing and follicle­stimulating hormones, decreased testosterone, and aspermia or hypo­spermia in 85% of LL patients but in only 25% of BL patients.
    7. LL patients may become impotent and infertile. Impotence is sometimes responsive to testosterone replacement.
    8. Amyloidosis Secondary Amyloidosis is a complication of LL leprosy and ENL that is encountered infrequently in the antibiotic era. This complication may result in abnormalities of hepatic and particularly renal function.

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