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DIFFERENTIAL DIAGNOSIS OF LEPROSY - ( H.D.)
THE DIFFERENTIAL DIAGNOSIS OF LEPROSY (H. D.)
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When I first started my work in caring for leprosy patients back in the 1950’s, W.H.O. estimated that, at the time, there were approx. 15 million people suffering from the disease with a prevalence rate as high as 260 per 10,000 in some areas, such as Tamil Nadu, in South India. W.H.O. now estimates that the figure could be as low as 1.8 million (in 1996), with a prevalence rate, in some areas, of only 1 per 10,000, which is the rate below which The World Health Organisation (WHO), from the year 2000, will declare that leprosy in that area is no longer a public health problem. Why the dramatic fall in the numbers in such a relatively brief space of time? Thanks go to those who have implemented Multi-Drug-Therapy (MDT). Even though certain areas, once endemic for leprosy, will no longer have a Leprosy Public Health Problem, the bacillus will remain in the area and medical personnel must be alert for H.D. signs and symptoms which need to be differentiated from many other leprosy-like conditions.
Of course, it has to be remembered that even though a person within the estimated 1.8 million is declared bacteriologically “negative” and removed from the clinic register, that person does not cease to be a leprosy sufferer if there has been nerve damage and consequent deformity, disability, blindness and rejection because of stigma. It is possible for that person to remain a leprosy suffer for the rest of his / her life. “Leprosy” therefore, is not a condition that can be determined by the presence of bacilli alone but also by the way the disease has affected that person in terms of human relationships. It stands to reason, therefore, that long after the estimated 1.8 million sufferers (in 1996) has been reduced to nil, (if that can ever be possible before we have an anti-leprosy vaccine - we still are detecting nearly 600,000 new cases a year - we shall still have patients in need of physiotherapy, reconstructive surgery, occupational therapy and rehabilitation. Please remember that EVERY HOUR, SIXTY FIVE NEW CASES OF LEPROSY ARE BEING DETECTED, OF WHOM FOUR ALREADY HAVE SEVERE DISABILITY AND ELEVEN ARE CHILDREN. !!
It is true that, with the help of Multi-Drug-Therapy (MDT), by which it is now possible to actually kill the bacillus of leprosy, with bactericidal drugs, many patients have been cured. Most leprosy patients have the Paucibacilliary form of the disease which requires only about 6 months of treatment (at least 2 years for Multibacilliary or Lepromatous types) and once the patient is placed on Rifampicin, within 48 hours, the leprosy bacilli (M.leprae) start to fragment and become non-viable - unable to transmit the disease to others. Because of these factors, we should have expected a fall in the prevalence of the disease. The question is raised .. “Do we forget all about leprosy when and if the world-wide prevalence falls below 1 in 10,000”? - Obviously NO.
Another reason for the fall in numbers is because, in the past, some patients were diagnosed as having leprosy (Hansen’s Disease - H.D.) when, in fact, they had other diseases very much resembling leprosy in terms of signs and symptoms. Because of this, it is of the utmost importance that we be able to diagnose those leprosy-like conditions which can be manifest in the skin and nerves. Really, because of the stigma, it is a crime to diagnose a person as having leprosy when, for example, that patient may have diabetic ulcers. Because there are approx. 30+ conditions which can mimic leprosy in its early and late stages, it is important that we be able to identify them and so detect / diagnose those with true H.D..
There are four Cardinal Signs of leprosy, AT LEAST TWO of which must be seen in a patient before we can safely diagnose that person as having leprosy:- (1)Hypopigmented, localised skin patches, - (2)Anaesthesia or sensory deficit, particularly of touch and temperature. - (3)Thickened nerves, particularly peripheral nerves. (4) Non-cultivable, acid-fast bacilli present in skin lesions and or nasal mucosa . However, in some of the early cases of leprosy, diagnosis can be very difficult and may be impossible unless biopsies of skin and nerve are taken.
Leprosy is primarily a disease of the nerves, particularly the COOLER, peripheral nerves. Secondarily, it affects the skin. Because of this, we shall first look at those diseases of the peripheral nerves which resemble leprosy (H.D.) --- (1) Vitamin B deficiency:- This is seen in undernourished children in particular and is known as Peripheral Neuritis. Loss of feeling in the lower limbs can sometimes be experienced by those suffering from Vitamin B12, resulting in lesions in the posterior column of the spinal cord. - (2) Hypertrophic Interstitial Neuritis:- This condition is very rare, resulting in a thickening of the peripheral nerves requiring a biopsy of the nerve to properly diagnose. - (3) Toxic Neuritis:- Patients working in paint factories or other heavy metal industries dealing with lead, arsenic etc., may develop a leprosy-like anaesthesia and paralysis. Careful recording of case histories is essential. (4) Syphilitic Neuritis :- This is another disease affecting the posterior column of the spinal cord, resulting in lesions at the site which produce sensory loss. Again, careful case history needs to be made and a V.D.R.L. test made. - (5) Primary Amyloidosis of the Nerves:- This condition is very rare requiring a nerve biopsy to differentiate. - (6) Traumatic Neuritis:- A careful recording of Case History may reveal physical injury to the nerve, perhaps through an accident. - (7) Diabetes Mellitus:- Many patients with ulcerated feet have been wrongly diagnosed as having leprosy because Peripheral Neuritis in diabetes can result in loss of feeling, particularly in the lower extremities which often produces trophic or plantar ulcers. A careful physical examination will reveal sugar in the urine and an increased level of blood sugar. - (8) Cervical Rib:- This is another rare condition which is caused by pressure on the brachial plexus due to the presence of an accessory rib. Patients may be seen with leprosy-like hands with the small muscles atrophied and with sensory deficit at the ulnar border of the hand. In order to correctly diagnose this condition ,an X-Ray needs to be taken of the cervical spine. (9) Over-Riding of the Ulnar Nerve:- This extremely rare abnormality can result in loss of feeling in the ulnar aspect of the hand due to the ulnar nerve tripping over the medial epicondyle when the forearm is flexed, causing physical trauma to the nerve. This condition can even result in atrophy of the small muscles of the hand if allowed to persist for too long. It is important to be careful in making clinical examinations. This condition is corrected by surgery. - (10) Congenital Absence of Pain:- In all my years involved with leprosy sufferers, I have seen only one such case - in India - where the victim gives the impression of having Tuberculoid leprosy with the loss of fingers and toes. Careful examination and case history taking may reveal others in the same family with similar sensory loss. - (12 ) Progressive Muscular Atrophy :- In this case, the small muscles of the hands and feet may appear wasted as in Tuberculoid leprosy. There is no loss of feeling in this bi-lateral condition. - (13) Bernhardt’s Syndrone:- The lateral Femoral Cutaneous nerve is traumatised by a localised lesion resulting in loss of feeling in the anterolateral aspect of the thigh. - (14) Syringomyelia :- Mainly confined to the tropical areas of the world, there is a leprosy-like atrophy of the muscles and a loss of pain and temperature sensation in the hand and forearm, although the feeling of touch remains. - (15) Carpal Tunnel Syndrome: This condition gives all the impression of Tuberculoid leprosy hand deformity affecting the median nerve at the wrist. - (16) Bell’s Palsy:- This leprosy-like condition which results from Facial Nerve involvement causing facial paralysis and lagophthalmos (eye cannot close fully).
Leprosy-like Skin Diseases that can be confused with true H.D. are firstly those with hypopigmented (non-raised) macules:- (1) Hypopigmented patches often seen on the faces of young children in the tropics, especially in malnourished communities where worm infestation and vitamin deficiency in the diet may be the cause. - (2) Vitiligo of Leukoderma :- While in true leprosy we see only a partial loss of pigment of the skin, in this condition, there is a total loss of pigment (skin is white) , although in the early stages of Leukoderma, the patches are hypopigmented and can be confused with indeterminate leprosy. There is no sensory deficit in the patches. - (3) Tinea Versicolor:- This is common in the tropics. The neck and trunk are the prime sites and the lesions generally are multiple and have no loss of sensation. Fungi can be seen under the microscope. - (4) Pityriasis Rosea : They show up as small oval patches on the trunk. The scaley lesions can be confused with leprosy but there is no loss of feeling. (5) Seborrhoeic Dermatitis:- This condition generally occurs on the flexor aspect of the elbow and knee. When in the scalp, dandruff is often associated and the greasy scales sometime resemble lesions of H.D.. There is no sensory loss. - (6) Scleroderma (Morphea):- Sometimes these hypopigmented patches of Scleroderma may be confused with leprosy masules. Usually there is no loss of sensation in these patches which feel tough to the touch. - (7)Reaction to Injury:- When the scar patches are hypopigmented they can resemble leprosy but often they are hyperpigmented. There is no loss of feeling. - (8) Naevus Anaemicus:- These hypopigmented patches, seen anywhere on the body, have been present from birth. Careful questioning when taking case history is essential . There is no anaethesia in the lesions. These are the non-raised skin lesions that can be confused with H.D.
Raised Skin Lesions are as follows:- (1) Granuloma Annulare:- Mostly the small ring-like patches are seen on the dorsum of the hand . Sensations are normal. - (2) Tinea Corporis:- These lesions, generally known as “Ringworm”, usually are present in the groin and waist area.. Unlike a leprosy patch, these are always itchy and the fungi can be seen under the microscope. There is no loss of sensations. (3)Syphilis:- This can mimic lepromatous leprosy , especially in the late, secondary stage but there is no sensory loss. - (3) Post Kalaazar Dermal Leishmaniasis:- Lesions are usually circumoral. L.D. bodies are seen in Leishman-stained slides. Sensations are preserved. (4) Psoriasis:- Silvery white patches have no sensory loss. - (5) Discoid Lupus Erythematosis:- Usually butterfly shaped and symetrically distributed over the face with no loss of feeling. - (6) Lupus Vulgaris (T.B. of skin) :- Common in the tropics and can resemble the Tuberculoid and Borderline types of leprosy. Feeling is well preserved. (7) Multiple Neurofibromatosis:- These are multiple, small nodules, which can cover the whole body. No M.leprae are seen in the nodules. - (8) Multiple Lipomatosis :- This condition can be confused with nodular lepromatous leprosy but no acid-fast M.leprae are seen under the microscope. (9) Post Kalaazar Dermal Leishmaniasis :- Nodular lesions resembling lepromatous leprosy contain no M.leprae but rather L.D. bodies. (10)Allergic Dermatitis, Hypothyroidism and Shiny, oily skin can be differentiated from lepromatous H.D., because skin smears are negative for M.leprae. (11) Diabetic Ulcers can often be confused with ulcers due to neglected leprosy. (12) Burgeis Disease, often resulting in gangrenous feet, can be confused with leprosy, particularly in Bangladesh where children from a very early age start smoking cigarettes.
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