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LEPROSY - Reactional States - Types 1 and 2
LEPROSY - REACTIONAL STATES - TYPES 1 and 2
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In our discussions through "Forum on Leprosy", it is obvious that there is considerable confusion concerning "Reactions". Some imagine the condition to be the reaction of the patient to certain drugs used to treat leprosy, such as Dapsone, Rifampicin and Clofazamine etc., in much the same way that some patients are said to be "allergic to Penicillin etc.." However, leprosy reaction or, as it is more commonly referred to - "Lepra Reaction" is quite unique. I shall try to explain this dilemma in as simple a language as possible.
We now speak of two main types of Lepra Reaction , namely - "Type 1" and "Type 2", although some leprologists, such as Coombs and Gell, who are more into research, want to more thoroughly deal with the issue, so say that there are four hypersensitivity reactions. So far as we are concerned, for more practical purposes, we say that there are two. In the early days, we used different terminology - "Lepra Reaction" - to cover both "Type 1" reaction and "Erythema Nodosum Leprosum (ENL)" to describe what is now called "Type 2". Actually, these two hypersensitivity reactions are due to the antigens released by the dead and dying leprosy bacilli - M.leprae. It may help to refer to Lepra Reaction as an "Allergic Reaction" in much the same way as certain persons are allergic to milk products, etc.. In this case, some leprosy patients are more allergic to the antigens of M.leprae.
It needs to be understood that the leprosy germs are harmless little creatures causing no real problem until, from their cell walls, they starts to release antigens (toxins). This happens when they begin to fragment due to old age or when they are attacked by treatment drugs. When the antigens are released, they react with the "T-Type" lymphocytes (defence cells). This takes place in association with a rapid change in the patient's degree of immunity.
"Type 1" Reaction is typically seen in those patients whose immunological response is UNSTABLE. In the immunology of leprosy, there is what we call the "Immunological Spectrum" with the Tuberculoid (TT) type at one end (or Pole) which, in charting, is seen at the left-side end. of the Spectrum and the Lepromatous (LL)type at the other end or "pole". For this reason, we refer to Tuberculoid and Lepromatous types as "Polar" or Immunologically "stable" . Between these two Polar types there is the Borderline type - "BB" in the middle. Between the middle (BB) and the left pole, (TT) there is the Borderline-Tuberculoid (BT) type. OUTSIDE the spectrum, generally placed to the left of TT, is the "Indeterminate" type of leprosy which can either resolve spontaneously - because of a very strong CMI - or it may evolve, or rather devolve, into the lepromatous form, for which reason, it needs to be very carefully watched. Not all cases of Indeterminate leprosy are "self-healing". It should be clearly understood that Borderline leprosy, whether it be BT, BB or BL, is associated with "immunological INSTABILITY".
If, under treatment, there is a rapid increase in cell mediated immunity (CMI), with a proliferation of "T-Type" lymphocytes, we refer to the reaction as "Up-grading" or "Reversal" reaction. The reason for this terminology is because the natural course of Borderline leprosy is DOWN towards the lepromatous end of the spectrum. Reversing that, turns the patient around in the direction of the Tuberculoid pole and - a cure.. More CMI means "Upgrading" or a "Reversal" of the normal trend. On the contrary, if there is a reduction of immune response (CMI), we speak of "Downgrading" towards the lepromatous pole.
If the Borderline patient has an immune response leaning towards the Tuberculoid end of the spectrum or "pole", we call that type of leprosy, "Borderline - Tuberculoid (BT)", and, in such cases, Reaction ("Upgrading / Reversal") is most likely to happen EARLY, during the first six months of treatment. On the other hand, the borderline case with a lower degree of immunity (less CMI)"Downgrades" towards the lepromatous pole. Reactions in such BL cases, generally begin to occur LATER than six months into the course of treatment.
Borderline Tuberculoid "Upgrading / Reversal" reactions can result in very severe nerve damage in the EARLY stages of the disease / treatment. One of the most frequent clinical signs of "Type 1" reaction is a rapidly progressive change in the skin lesions, which become erythematous (reddish), shiny, slightly raised / prominent, warmer and with the appearance of erysipelas. Also, as with all aspects of signs and symptoms of leprosy in the left (Tuberculoid) side of the immunological spectrum, the lesions are LOCALISED and never bilateral. In more severe cases, necrosis of the lesion may result with some ulceration.
"Type 1" reaction can be very deceptive, with new crops of raised, shiny red lesions developing and giving the impression of "downgrading". Another false indication that "downgrading" is taking place (when actually this is "upgrading", leading towards a cure, because it is part of the healing process), is because the condition is accompanied by peripheral (cooler) nerve involvement, including much severe pain, tenderness and swelling especially where the nerve is close to the surface (cooler) . Common sites for this nerve involvement are the elbow - "funny-bone" (ulnar nerve) and the wrist (median nerve) where the nerves may be further traumatised because they passes by or near bony tunnels. Often the nerves have been so traumatised that they need to be carefully padded with cotton-wool and the arm placed in a sling. In the most severe cases, the nerve sheath needs to be excised to release the intraneural pressure caused by oedema and granuloma formations. Occasionally, but rarely, the nerve may be further traumatised when an abscess forms, creating a situation of extreme pain. I have heard ill-informed people say - "Oh, leprosy patients have a disease which does not inconvenience them too much because they cannot feel pain"!!! Nothing could be further from the truth. I have had "Type 1" reaction patients so distressed by their pain that they have pleaded with me to kill them to put them out of their misery. Their condition was further compounded by related feverish oedema of hands and feet with skin that occasionally came off like a rubber glove!
In Borderline Tuberculoid leprosy, there always is a great danger of motor nerve involvement, especially of the ulnar nerve, leading to "clawed-hand" deformity, being the most common deformity associated with "Type 1" reaction. The lateral popliteal nerve at the back of the knee, also needs to be carefully monitored because its reactional involvement can result in a "dropped-foot" and the facial-nerve, if involved, can result in a Bell's Palsy-like facial paralysis. For these reasons, it is so important that leprosy patients be carefully treated and have easy access to clinics where they may receive help in times of reaction.
This study is only on a very simple level but, if you want to know the histological changes that can result from "Type 1" reaction, please refer to Ridley's or Jopling's text books which are available from
AMAZON.COM Cyberspacial Book-shop. Some have asked - "Why go to all this trouble to explain the difference between "Type 1" and "Type 2" reactional states "? Lepra Reaction in H.D. is an enigma. Although, paradoxically, "Type 1" is part of the healing process - an "Up-Grading" of the body's immune response or a "reversal" of the disease's natural process - the impression it gives is that the disease is increasing and that the patient is not responding to medication. For this reason, some medical workers have stopped all treatment and some patients have refused to take their medication, believing that it is the treatment that is causing their fever and malaise. Health Education and Prevention of Disability teaching is, therefore, of vital importance, especially for those prone to reaction.
TYPE 2 REACTION . In relation to Lepra Reaction, it is important to remember that the two types of Immunity are involved - "Humoral Antibody Response" and "Cell Mediated Immunity (CMI)". Whereas "Type 1" reaction in B.T. cases first manifests in the early stages of treatment, generally within the first six months of treatment, "Type 2" (Erythema Nodosum Leprosum or ENL) reaction, first occurs LATER in the course of treatment. As with all aspects of the disease nearer to the lepromatous pole of the immunological spectrum, the reactional lesions, plaques or nodules appear bilaterally and symetrically , differing from "Type 1" which are localised.
"Type 2" may manifest itself on the face, medial aspects of the thighs and the forearms. In fact, the lesions may appear on ANY area of the skin, except the warmer regions such as scalp, groins, perineum etc.. Occasionally, they may appear even on the palms and soles of the feet. Some of these ENL lesions my rupture and become necrotic. "Type 2" reaction may also be accompanied by severe eye problems, especially Iritis which may be confused with conjunctivitis. The testes (cooler) may be swollen and extremely tender. There may be softening of the bones, particularly in hands, feet and tibiae. Muscle pain (Myositis) and swelling of the joints give the impression of severe rheumatism. Borderline Lepromatous (B.L.) reactional patients suffer in the extreme and their condition may be triggered off by several factors - the trauma of surgery, physical and mental stress, protective inoculations and even pregnancy. For a more detailed study of these Reactional States, please access books by Bryceson, Ridley and Jopling, available from AMAZON.COM, as above. If you wish, please email us at :- keithskilli@ozemail.com.au
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