Day 9: Leprosy and Karigiri

The day started off with a talk on Leprosy, the WHO has given cardinal features to consider leprosy in endemic areas; the idea being easy for health care providers to recognize.

1.     Anaesthetic skin lesion

2.     Enlargement of peripheral nerves with evidence of nerve damage (loss of sensation, weakness, paralysis)

3.     Positive skin smears

The above sounds simple enough but it can be difficult to diagnosis, patient can have neuropathy only, papules, plaques, ulcers, loss of hair (eye brows or eye lashes), and ichthyosis.

The talk further highlighted the spectrum of leprosy from tuberculoid to lepromatous and the in between. The disease further is classified based on paucibacillary or multi-bacillary leprosy which will alter the drug regimen, and the duration.

India has around 60% of the worlds leprosy (together with Indonesia and Brazil make up 80% of the worlds leprosy cases) and it is still very much prevalent in certain areas. Interestingly, it is considered eliminated in India but there are pockets of areas where >1000 cases are diagnosed yearly. In the afternoon we had a field trip to Karigiri hospital (http://karigiri.org/blogs/) which gives a multi-disciplinary approach to patients living with leprosy in addition to conducting research. The take home message – the disease can be easily treated if diagnosed early, but can cause significant morbidity, and a decrease in quality of life is diagnosed late.

We still do not know why some people develop tuberculoid leprosy (TH1 response, granulomas), vs lepromatous (TH2, poor response and lesions are usually full of bacilli). The incubation period is suspected to be around 5 years, but up to 20. It is believes to be transmitted by respiratory route, but likely prolonged exposure. During exposure the majority of the time are body will be able to deal with it (similar to tuberculosis). Commonly, patients present with just a hypaesthesic patch, hypo or hyperpigmented.

Differentials of this are great: syphilis, leishmaniasis (post kala azar), tinea versicolor, sarcoid, vitiligo, post inflammatory skin hypopigmentation.

WHO classification:

Pauci-bacillary leprosy – up to 5 skin lesions or 4 and 1 nerve trunk

Multi-bacillary leprosy: More than 5 skin lesions, skin more than 1 nerve trunk or skin smear positive

Two reactions termed type 1 (or reverse) and type 2 (ENL), can occur at any point during therapy, even before or after. Type 1 is where lesions worsen, more often with tuberculoid leprosy, if severe, steroids are given. Type 2 is an arthrus reaction, systemically unwell, enlarged lymph nodes, painful nodules appearing, often treated with thalidomide +/- steroids.

Treatment:

Paucibacillary: 6 months with dapsone daily, rifampicin monthly, should finish therapy within 9 months.

Multibacillary: 12 months of daily dapsone and rifampicin monthly , and daily clofazimine 300 monthly or 50 mg daily.

 (within 18 months or start again)

At Karigiri hospital, we split up into groups of 4 and got a great tour of the facilities. Our first talk was with an orthopedic surgeon he explained some of the common neuropathies with leprosy. Generally, it will be nerves that are superficial, near joints – commonly ulnar, median in the upper extremity, and common fibular and posterior tibial nerve in lower extremities.

Features of nerve loss:

1.     Sensory loss – possibly can injury themselves

2.     Motor loss: functional impairment

3.     autonomics: issues with sweating, leading to dry skin and ulcers

He explained that with claw hand from ulnar nerve issues, patients will have severe limitations gripping things (making a fist), ape hand from median nerve injury causes wasting of thenar muscles and issues abducting and opposing thumb – thus making writing or pinching impossible.

Patient with injury to posterior tibial nerve, lack sensation on most of the bottom of the foot, also cannot plantar flex, where deep peroneal nerve injury causes high stepping gait and can often drag foot.

Patient are generally given a trial of steroids to see if neuropathy can improve, but if left with significant impairment, can be candidates for tendon transfers.  We saw some post op patients and the results were very impressive.

We then saw a variety of clinic patients who were very receptive to us examining them. Many just presented with a hypoaesthetic skin patch, others had significant disability. Patients get assessed initially by a dermatologist, they get their lesions biopsied but generally initially get skin snips around the face (ear lobe, chin), too look for bacilli for classification. After, we moved on to the physiotherapy and occupational therapy station where they showed us the type some the devices they have to help people. I was very impressed by some of the devices they had made to help people in the community.

Hypoesthetic patch on patient's back

This patient was kind enough to allow us to use his picture, classic leonine faces in lepromatous leprosy, he also has madarosis (missing eye lashes), and missing eyebrows.

Patient with significant neuropathy.

side of patient’s face with lepromatous leprosy, missing the bulk of facial hair.