LEPRA - REACTIONAL TYPES ONE AND TWO




"TYPE ONE" LEPRA REACTION - Continuation of "Reactions - how to manage ".



This is often called a "delayed sensitivity reaction", during which there is a fight going on between the M.leprae antigens and the "T - type" lymphocytes, producing a rapid "upgrading" change in CMI. It is seen mainly in BT cases under treatment. It is important to closely observe BT and BB patients for the first 6 months after starting MDT because that is when serious Type 1 reaction can occur, resulting in sudden paralysis and deformity. BT patients are particularly vulnerable and need to be carefully monitored for the first few months after initiating MDT. Treatment. The main clinical features are :- erythema and swelling of leprosy skin lesions - all or some. And new lesions also may appear, being shiny, warm to the touch and raised. In the mid-range Borderline BB type of reaction, at first it may be difficult to know whether it is one of "down-grading" or "up-grading". That can only really be determined through histology and also by lepromin testing which indicates if there is an improved immunological response. If there is, then it will be a "up-grading" reaction. The word to remember in relation to Type 1, is "rapid" and involves peripheral nerves which may suddenly become swollen, with extreme pain, especially where the nerve is most superficial (COOLER). As sudden as severe signs are manifest, so we need to administer Prednisolone with no time wasted. What causes such intense pain? It is due to intraneural pressure of oedema, along with cellular reactions (granuloma formation). In fact, intraneural pressure is what is causing most of the trouble. Even when there is no reaction, when such immunological response pressure builds up within the nerve sheath, the small blood vessels which supply nourishment (oxygen) to the nerves, are strangled, thus decreasing or cutting off the blood supply. Where the sensory nerves are starved or affected in this way, through anoxia, sensations are lost. Where the motor nerves are deprived of nourishment, paralysis may result, and where the autonomic nerves suffer from anoxia, (lack of oxygen), the sweat / sebaceous glands and hair follicles, along with blood vessels are adversely affected. In Type 1 reaction, the motor-nerves seem more at risk, particularly -- (1) the ulnar, in BT cases,and where pain is experienced in the elbow region, resulting in a "clawed" little finger . (2) - The lateral popliteal nerve at the back of the knee also is at risk, resulting in "dropped foot", again, more especially in BT cases. (3) - Patients with a lesion on the cheek need to be watched because they are prone to develop Type One reaction affecting the Facial Nerve, resulting in facial palsy. If neglected, these deformities may become permanent, however, with correct treatment and very careful nursing, such paralyses may possibly be reversed. In Type One reaction, oedema may also be manifest in the hands/palms, feet /soles and face. Often, oedema of hands and feet may indicate that Type 1 reaction is developing. Paradoxically, in a patient with Type One, "up-grading" reaction, especially if there is fever and malaise (less in Type 1, as compared with Type 2) the patient may become depressed, imagining that the disease is progressing (getting worse) because new lesions may appear, even though the patient is on the upward road to healing, with lepromin tests showing that the immune response is becoming more positive.


In Type One reaction, histological testing of the dermis shows a reduction in the number of M.leprae bacilli, while there is an increase in the number of defence cells such as lymphocytes, epithelioid and giant cells. It is just the opposite in "down-grading" Type 2 , with the number of M.leprae bacilli on the increase and the defence cells being replaced by macrophages. Watch out for oedema of extremities, along with nerve pain and swelling (neuritis). Sometimes, in order to save the nerve from serious damage, the oedema has to be reduced by excising the nerve sheath. The swollen, tender nerves also need to be carefully protected with pads of cotton wool. There also could be loss of sensations and muscular paralysis. Histological features in "reversal" or Type 1 reaction are oedema, reduction of M.leprae but increase of defence cells such as "T-type" lymphocytes, epithelioid cells, giant cells.



"TYPE 2 " LEPRA REACTION



This "down-grading" reaction is not associated with a change in CMI because it is a humoral-antibody-response. It is also called E.N.L. (Erythema Nodosum Leprosum) associated with severe bone and joint pain, fever, neuritis, malaise, rhinitis, orchitis, iritis, orchitis, lymphadenitis, epistaxis , oedema and other conditions. E.N.L. is a very serious condition, having most traumatic repercussions. It can even result in softening and absorption of the bones. Fingers need to be encased in plastic splints to retain their shape during bouts of ENL. In exacerbated cases of ENL, lesions may become vesicular and rupture with erythema necroticans.


TREATMENT / CONTROL - is most effective with Prednisolone - 40 to 80 mg. Per day - single dose., gradually reduced. In cases where corticosteroids fail to give relief and the patient is in a very desperate situation, Thalidomide is the anti-inflammatory drug of choice , used only in Type 2 reactions = 400 mg. daily until the reaction has been brought under control and then , as with Prednisolone, gradually reduced . Because of the controversial (teratogenic effects) nature of this drug, it must never be given to pre-menopausal women unless they have had a hysterectomy / tubectomy. Sometimes the neuritic pain of lepra reactions is so severe that intraneural injections of prednisolone are given and also lignocaine. I have heard it said -- "Oh, leprosy patients don't feel pain !!!!!!




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