Wednesday, November 9, 2011

Leprosy in Ecuador (clinical cases)


Leprosy Mailing List – October 5th, 2011 
Ref.:   Leprosy in Ecuador (clinical cases)
From: G. Warren, Sydney,  Australia

Dear Dr Verduga,
What an excellent collection of photos (LML September 27th, 2011), and the variety of Borderline manifestations and reactions is a lovely example for others who live in areas where natural skin colours vary. 
Cases 1 and 2 (see attachment) which are on slightly darker skin and show excellently the extra erythema and swelling that so often occurs is the reactive phase. 
In case 1 the central immune area can be missed on the arm but is evident on the back of the elbow.
Case 2 also shows how definite can be the inner edge of the lesions in BB. Here the true borderline rings with their central immune area can be appreciated.
The case number 3 is obviously a lighter skin initially and the leprosy lesions are not really reactive and so show a lot less than the lesions in cases 1 and 2 which are on slightly darker skin
What a lovely teaching slides.  In all cases the severity of reaction needs to be watched carefully so that if there is any evidence of acute nerve involvement steroids can be given in adequate dosage.  However case 3 needs to be watched as carefully as those already in reaction, when therapy is started.
I normally do not give steroids for all BB in reaction unless the lesions are on the face or there is definite evidence of nerve damage.  When lesions are on the face and particularly around the eye the underline facial nerve and its branches are at risk and, I do not want to produce a facial palsy.
The beginning of the treatment with rifampicin and dapsone may well precipitate acute nerve deterioration as the drugs kill more bacilli and so cause a rapid rise in the amount of the immune response to the antigen freed from the dead bacilli.  This in  turn increases the swelling inside the nerve sheath and so the risk of nerve damage.  This response stimulates more reaction and hence swelling in the skin lesions as well as possibly a neural deficit. 
I like to give clofazimine initially as it has an anti-reaction effect as well it as bacteriostatic and bactericidal effect.  It is generally taught that clofazimine is effective in preventing Type 2 ( ENL) reaction.  Yes that is correct, that is where it is most used.  But when doing the initial drug trials we often used it in patients with Type 1 reaction as well and it also is helpful for that.  It may not be as bactericidal as some other drugs but it is adequate to prevent increasing numbers of bacilli as it is bacteriostatic.  What is significant is that many BL/BB  type patients develop both Type 1 and Type 2 reaction.  Especially when inter-current infections and or anaemia and other metabolic diseases are dealt with.  So with patients who have those unstable forms of  BL/BB  leprosy it is worth giving clofazimine alone till the other medical problems are dealt with so that as the Bodies natural destruction of M. leprae  occurs the rise in T sensitised lymphocytes will not result in a Type 1. reaction. 
In  a patient with BL/BB leprosy the lesions may appear very vague initially and so it is difficult to tell if there is a borderline element.  In these patients (and in some others) it is most likely that once the patient’s own ability to fight the disease rises, and the number of dead bacilli rises, so the number of leprosy antigens rises- the body will produce both “T and “B” lymphocytes.  The development of clinical evidence of Type 1 or type 2 reaction will depend on, many factors.  It was fascinating to see that patients with BL/BB leprosy with some evidence of Type 1 reaction would, if given an anti-smalll pox vaccination, go into Type 2 reaction!  Yes that was in the late 1960s when we needed to give the smallpox but I hated doing it as so many patients of all immune levels, went into reaction.  But the use of clofazimine for these patients improved their ability to cope.  So this is just one of the reasons why I like any patient whom I suspect of being Reaction prone, to start on clofazimine alone for the first 6-8 weeks.  It certainly reduced the problems in the lighter skinned patients with other medical problems as well.
Again commenting about the case number 3 (see attachment) it shows very well BL lesions that could well go into reaction on full MDT- the nerve damage can still occur so we need to watch but please do not give steroids for many months duration - should not be needed.
These are the patients I found did excellently on clofazimine initially and then once the tendency to reaction has settled put onto full MDT.  Yes, by all means give MDT in full dose once things look more stable.
Cases number 4 and 5 have a reported smear of 3+.  Case number 5 is typical, a lovely one of nodules on ear lobes, I would love to see pictures of this body or limbs as often the nodules on the ears precede obvious lesions elsewhere and are missed are being LL. 
I find clofazimine the best to start treatment as rifampicin does seem to speed up the development of ENL!  Patients with a high bacteriological index (BI) and especially a high morphological index (MI)  need more than the 12 month multi-bacillary multi-drug therapy (MB-MDT) suggested by WHO.  Sorry but the number of BL and LL patients I have seen who are still very active after 12 months MDT tells me that they are those who must have these drugs for longer than recommended if we are to prevent the disease reappearing in a few years time.
My long term experience shows that even with two or three drugs the patients with severe LL/BL leprosy need more than 12 months to be really cleared.  The bacilli live in the nerves and even rifampicin cannot get at them there. 
You asked for comments.  Fine I do not mind and hope this helps you in your general programme.  I have been treating leprosy including the reconstructive surgery since 1959 and if I can help by email please feel you can write to me.<grace.warren@levelthirteen.net>.  I was Medical Superintendent of the Hong Kong leprosy Hospital from 1960 to 1975 when it closed and after that taught in many countries round the world.
With Best wishes in your programme,
Grace Warren.

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