Lepra Reaction And Erythma Nodosum Leprosum

Lepra Reaction And Erythma Nodosum Leprosum


 Type IReversal reaction/lepra reaction /Jopling Type I 

  • Type lV  delayed hypersensitivity.
  • lf precede therapy than termed down grading reaction i.e. towards LL.
  • Occur after therapy than termed reversal  reaction i.e. towards more tuberculoid.
  • Timing of occurrence is early
  • Common in patients between the two poles of the leprosy spectrum with immunologically unstable borderline forms
  • Higher incidence in BB and BL patients as compared with BT patients.

 Manifestations

  • Inflammation within previously involved macules,papules and plaques
  • Skin lesions suddenly becomes reddish, swollen, warm, painful and tender
  • The rest of skin is normal
  • Neuritis with loss of nerve function
  • Weakness of eyelid closure other organs not affected
  • MC nerve trunk involved – Ulnar at elbow
  • Most characteristic microscopic feature of type I reaction is Edema.

 Cytokines involved

  • Increased activity of T helper (Th)-1 lymphocytes expressing IL-2 and IFN-γ
  • IL-12 is consistently expressed and IL-4 is absent.
  • The IFN-γ and TNF-α producing CD4 cells and T cytotoxic cells are selectively increased .

 Treatment:

Treatment of lepra reactions according to severity 

 

Type 1

Type 2

Mild

NSAIDS

NSAIDS

Moderate

Nsaids , Corticosteroids

NSAIDS , Clofazimine, Thalidomide, Chloroquine

Severe

NSAIDS . Corticosteroids

Thalidomide , Corticosteroids , Parenteral antimony

 
  • DOC glucocorticoids
  • Clofazimine also given
  • Thalidomide – ineffective.
  • Irreversible nerve damage  can set in less than 24 hours
  • So treat promptly with steroids
  • Foot drop d/t involvement of common peroneal nerve

Type II lepra reaction – Erythema Nodosum Leproticum/Jopling Type II:

  • Type II hypersensitivity reaction occurs exclusively in BL, LL .
  • Usually follows therapy (sulfone syndrome) but may precede therapy.

Manifestations

  • Red, painful, tender, cutaneous nodules (deep) appear that are not associated with leprosy patches. 
  • ENL may appear commonly on face, arms, legs. 
  • painful erythematous papules that resolve spontaneously in few day to weeks
  • May reoccur.
  • Iritis

May effect other organ like:

  1.  Periosteal pain (especially tibiae)
  2.  myositis
  3.  pain and swelling of the tendons and joints
  4.  rhinitis, epistaxi
  5.   painful dactylitis
  6.  swollen tender lymph nodes especially femoral
  7.   acute epididymo-orchitis,
  8.  hepato-splenomegaly with hepatitis
  9.   endocarditis with/without arhythmia
  10.  Glomerulonephritis.

  Central role in pathobiology:

  • TNF
  • Tumor necrosis factor-alpha play a central role in the pathobiology of type II lepra reaction/ ENL.
  • A predominant Th2 cytokine profile has been observed  with increased expression of IL-6, IL-8, and IL-10 as well as sustained production of Th2 cytokines, IL-4, and IL-5. 

Treatment

  • Mild – antipyretics alone
  • Moderate to severe – Ist drug to be used glucocorticoids
  • DOC: thalidomide
  • Clofazimine – More active than in Type I.

 Lucio’s Phenomenon:

  • Type  III Hypersensitivity exclusively in diffuse lepromatosis form of LL,
  • Usually seen in untreated patient. .

 Treatment:

  • Glucocorticoid and thalidomide is effective.
  • Wound care and therapy for bacteremia.
Exam Question
 

 Type I Reversal reaction/lepra reaction /Jopling Type I

 Type lV  delayed hypersensitivity.

  • Common in patients between the two poles of the leprosy spectrum with immunologically unstable borderline forms
  • Higher incidence in BB and BL patients as compared with BT patients.

 Manifestations

  • Inflammation within previously involved macules,papules and plaques
  • Skin lesions suddenly becomes reddish, swollen, warm, painful and tender
  • The rest of skin is normal
  • MC nerve trunk involved – Ulnar at elbow
  • Most characteristic microscopic feature of type I reaction is Edema.

 Cytokines involved

  • Increased activity of T helper (Th)-1 lymphocytes expressing IL-2 and IFN-γ

 Treatment:

Treatment of lepra reactions according to severity 

 

Type 1

Type 2

Mild

NSAIDS

NSAIDS

Moderate

Nsaids , Corticosteroids

NSAIDS , Clofazimine, Thalidomide, Chloroquine

Severe

NSAIDS . Corticosteroids

Thalidomide , Corticosteroids , Parenteral antimony

 
  •  DOC glucocorticoids
  •  Clofazimine also given
  •  Thalidomide – ineffective.
  •  Irreversible nerve damage  can set in less than 24 hours
  •  So treat promptly with steroids
  •  Foot drop d/t involvement of common peroneal nerve

Type II lepra reaction – Erythema Nodosum Leproticum/Jopling Type II:

  • Type III hypersensitivity reaction occurs exclusively in BL, LL .
  • Usually follows therapy (sulfone syndrome) but may precede therapy.

Manifestations

  • Red, painful, tender, cutaneous nodules (deep) appear that are not associated with leprosy patches. 
  • ENL may appear commonly on face, arms, legs. 

  Central role in pathobiology:

  • TNF
  • Tumor necrosis factor-alpha play a central role in the pathobiology of type II lepra reaction/ ENL. 

Treatment

  •  Mild – antipyretics alone
  •  Moderate to severe – Ist drug to be used glucocorticoids
  •  DOC: thalidomide
  •  Clofazimine – More active than in Type I.
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