Tuesday, April 10, 2012

Clinical case. Borderline leprosy in reaction in a boy


 Leprosy Mailing List – April 6th, 2012 
Ref.:   Clinical case. Borderline leprosy in reaction in a boy.From: Warren G., Melbourne, Australia

Dear Dr Noto,
I would like to congratulate Dr Barreto and Dr Cabral on the excellent case presentation (LML 24 March 2012) that opens the way to discussion of several important points.  There were some interesting picture of biopsies, but one wonders what type of lesions were biopsied.  One would expect those type of pictures from the BT end of spectrum but I wonder if any biopsy was taken from a vague lesion?
1. The lesions appeared at age 4 with no known contacts of leprosy, but we do not know the incidence of leprosy in the area or if the patient had lived elsewhere.  The state of Mato Grosso is well known for his leprosy endemicity.  Was any (extended) contact examination done?

2. The condition was diagnosed as eczema at several occasions, by different health staff and even by the local leprosy reference centre.  It does accent in endemic areas that any skin lesion not reacting to normal treatment needs to be followed up and the possibility of leprosy should be taken into account.  Even the local leprosy clinic did not think about this possibility.  This is an aftermath we are seeing in many place now that the statement that leprosy is eliminated has resulted in many early cases being missed.  WHAT WE DO NOT LOOK FOR WE WILL NEVER SEE!  One should be highly suspicious of eczema that continued for twelve months not reacting to treatment.
3. I am interested in the degree of affection of the nerves.  There is no mention of sensory changes (in the lesions, hands and feet).  Perhaps a little hard  to test, in a 5 year old. The history implies no motor nerve deficit, but the biopsy showed bacilli in nerves and some nerves were easily palpable and visible.  The use of steroids  is certainly indicated.  We see that the inflammation has settled clinically in 2 months so hopefully he should not develop further nerve involvement.  The steroids cannot reverse any real damage to the actual fibres that has occurred, but there is no point in continuing the steroids as their job is to reduce the inflammation.  However, it will not encourage regrowth of damaged fibres. 
4. Long experience with dozens of patient with this type of reaction has shown me that 10-12 weeks of steroids is all that most need and then the continued use of MDT, possibly with extra clofazimine to prevent and control further reaction.  I certainly did find that in lepra reaction in the BL/BBish type one often had both types of reaction at the same time (acute redness and even ulceration of the lesions that looked real BB/BT and ENL on the BL/LL ones).  In the pictures of the patient in question there is definitely a suggestion of BLish type lesions on wrists and also the ear lobe) and even around the knees.  Yes, he has downgraded, but lesions now are right across the spectrum and I would not be surprised if there was some early ENL in those arm and face (ear lobe) lesions or, that ENL comes once the steroid is discontinued.  Hence the suggestion that extra clofazimine may be of help. 
5. Steroids are often continued for a prolonged period and does a lot of harm to the patient’s own metabolism.  I have seen too many patients die because they had had long periods of steroids and for various reasons they were not restarted when medical complications arose (e.g. one teenage boy got a severe flue 6 months after 5 years of steroids stopped and he did not get adequate medical care and he died within a week). 
6. The other problem that is often forgotten is that steroids are effective  in preventing inflammation.  At the same time, the body’s own abilities to control the infection are slowed down by the steroids and this affects the ability of the body to reduce the degree of infection caused by M. leprae.  In fact in a well controlled drug trial severe LL patients were given Rifampycin daily under supervision for 5 years.  After that time the disease appeared controlled, but nerve biopsy and culture revealed M. leprae that were fully sensitive to the Rifampycin.  It is now accepted that the antibiotics while being able to kill the bacteria in the blood and other tissues, but cannot eliminate them from the nerves.  Once MDT is completed the bacteria come out, start multiplying again and the patient will relapse in years time. Hence, once definite durations for MDT were suggested by W.H.O., we  started counting the months recommended  as those not on steroids.  If a patient had 3 months steroids he had 24 plus 3 months of MDT for Multibacillary leprosy.  This certainly seemed to prevent many relapses.  In patients with less natural resistance ie the LL/BL type of disease that is well developed at diagnosis, I feel they need longer MDT than that recommended by W.H.O. to ensure no relapse. 
7. Hence I hope that the boy affected will have at least twelve months full MDT after the steroids are stopped.  He is obviously borderline in type and so should have some ability to  eliminate and control the infection. 
8. I am interested to see if he was given tricyclics.  Yes, I use them a lot on adults and think they are excellent in helping to minimise tension and fear of the disease.  However, I must confess  I rarely give tricyclics to small children though have frequently use Phenobarb with good results.  They  can help reduce the duration that one needs to give the steroids.
I do hope your presentation will encourage others to look more carefully for diagnosisand use steroids wisely.
Yours sincerely,
Grace Warren
Previously  Superintendent Hong Kong Leprosarium ( 1960-75)
Advisor in leprosy and   Reconstructive surgery in Asia  ( 1975-95)

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