On tabulating the data of 50 patients of hand deformities the following results were obtained: 44 cases (88%) had claw hands (complete claw hand: 30 cases, ulnar claw hand: 14 cases); 26 cases had absorption of fingers (52%); 16 cases had gross callosities (32.64%); 10 cases had frozen hands (18.51%); 8 cases had traumatic/ trophic ulcers (16%); 8 cases had banana fingers (16%); 6 cases had twisted fingers (12%). All 50 cases showed marked anaesthesia along with thenar and hypothenar wasting. Similarly, the data of 50 patients of foot deformities is as follows: 42 cases had foot drop (84%); 42 cases had absorption of toes (84%); 34 cases had hammer toes (68%); 34 cases had trophic ulcers (68%); 32 cases had flat foot deformity (64%); 30 cases had pseudo-ainhum deformity (60%); 18 cases had twisted toes (36%) and all the cases showed marked anaesthesia of the limb.

There was no case with only a solitary deformity of either hand or foot. Every case had a combination of multiple deformities. Along with the above mentioned deformities, a few cases had some associated features as well. 1 male had evident left eye lagophthalmos and right-side facial palsy and another male showed gross unilateral left sided upper limb lymphedema. 2 females had undergone a below-knee amputation owing to chronic trophic ulcers and subsequent osteomyelitis and 1 male had vitiliginous patches on the extensor surfaces of both legs.

Discussion

Leprosy or Hansen’s disease has been termed Kushta rog in India. The word ‘kushta’ is derived from a Sanskrit word ‘kushnati’ which means, ‘eating away’ [4]. Leprosy has a protracted course with several cutaneous and systemic features. The cutaneous alterations can range from a solitary hypo pigmented, hypo-anaesthetic patch to widespread multiple infiltrative lesions. The bacterial propensity to invade peripheral nerves is the cause of anaesthesia and loss of motor function which forms the mainstay of deformities, (Figure 1).

Figure 1: Bar graph of age and sex distribution of cases.

Even after years of relentless efforts, we have not been able to culture the bacillus on an artificial medium. Mouse foot pads and other animal models like ninebanded Armadillo and Mangabey monkeys etc. are still being used for culture [5]. The cutaneous manifestations and associated co-morbidities cause of severe distress. Leprosy has been stigmatised since long which raises misery and psychological impact on patients, (Figure 2).

Figure 2: Pie diagram showing the distribution of deformities of hands and feet.

M. leprae does not follow the famous Koch’s postulates in many ways [6]. It is the only bacterium known to affect the peripheral nerves. Once leprosy sets in, the accompanying deformities tend to cause marked impairment of function, (Figure 3).

Figure 3: Photograph of a case of B/L complete claw hand with prominent muscle wasting.

Leprosy presents as a spectrum based on the immunological susceptibility of the individual. The WHO classification of Pauci Bacillary (PB) and Multi Bacillary (MB) disease is based on the number of patches on the body. The ‘Ridley and Jopling classification scheme’ is most widely accepted in practice. Leprosy has been classified into 5 types as per this classification: 1. Tuberculoid (TT), 2. Borderline Tuberculoid (BT), 3. Mid- Borderline (BB), 4. Borderline Lepromatous (BL) 5. Lepromatous (LL). The BL and LL forms of Leprosy are multibacillary which may culminate into deformities if left untreated. Several other classification systems have been proposed with few additional advantages. The Indian classification has highlighted the two distinct forms of Leprosy: Indeterminate and Pure neuritic [7].

The advent of multidrug therapy in 1981 in India has been of definite aid in reducing the incidence and burden of Leprosy. The Enhanced Global Strategy was launched by WHO in 2009, with an aim to decrease the disease burden of leprosy from 2011-2015, targeting to bring down the number of new cases with Grade-2 Disability (G2D) per 1 lakh population by a minimum of 35% [8].

The World Health Organisation has proposed the disability grading in 1970: (For deformities of hands and feet):

• Grade 0: No anaesthesia; no visible deformity or change.

• Grade 1: Anaesthesia present, but no visible damage or deformity.

• Grade 2: Visible deformity or damage present.

Furthermore, the definitions of Disability and Deformity have independent distinction [9].

Definition of disability

Lack of ability to perform an activity considered normal for a human being of the same age, gender and culture.

Definition of deformity

Visible alteration in the form, shape or appearance of body due to impairment produced by the disease process.

Deformities can be further classified as primary and secondary deformities. Primary deformity results due to the direct consequence of disease process (e.g. ciliary and superciliary madarosis, muscle wasting, gynecomastia etc.). Secondary deformity cannot be directly linked to the primary pathology. It usually occurs as a consequence of the primary deformity. (e.g. Trophic ulcers, foot drop, callosities etc.).

Deformities of the hands and feet can be further subclassified as follows [9], (Figure 4).

Figure 4: Photograph depicting the Banana finger deformity.

Deformities of the hand

Specific deformities: Reaction hand, frozen hands, twisted fingers, Intrinsic plus fingers, Banana fingers.

Motor paralytic deformities: Ulnar claw hand; ulnarmedian claw hand; wrist drop; ‘z’ thumb and weak pinch; reversal of distal transverse metacarpal arch; interphalangeal joint contracture of the thumb.

Anaesthetic deformities: Anaesthesia.

Deformities of the feet

Specific deformities: Frozen foot, twisted toes.

Motor paralytic deformities: Claw toes, foot droppartial or complete.

Anaesthetic deformities: Ulcers, absorption, disorganization of foot.

Three important factors that play a role in the causation of deformities are [10]

• Direct invasion by mycobacterium leprae.

• Muscle damage and wasting, secondary to motor paralysis.

• Impaired sensation and anaesthesia which result in trivial wounds going unnoticed and thereby leading to the development of trophic ulcers.

Factors that make an individual prone to get deformities

Age: Middle aged individuals are more susceptible

Sex: Males are at a higher risk than females.

Duration: Longer duration of active disease can result in deformities.

Type of disease and the immune status of the patient: Poor immunity, immunosuppressive therapy and the lepromatous spectrum of the disease are risk factors.

Occupation: Occupation of the patient which can cause trauma to an accessible anaesthetic part is an important risk factor.

Patient attitude and concerns: Inappropriate attitude towards the occurrence of deformity, can lead to severe damage. Carelessness can arise out of intense apathy for the disease.

Treatment: Inadequate and improper treatment can result in persistence of the disease with the on-going process culminating into deformities.

Nerve paralysis: Nerve paralysis can occur with preceding signs of neuritis, but if it occurs silently without any evident signs, then the loss of function and sensation may go unnoticed and cause impairment.

Experts in Bangalore studied 50 cases of Leprosy. Grade 2 deformity was found to be predominant, which is consistent with the finding in our study. Claw hands and trophic ulcers were seen in 38% of cases [11]. In our study we found that claw hands were seen in 88% cases and tropic ulcers were seen in 68% cases, (Figure 5).

Figure 5: B/L complete claw hand with twisted fingers. A small bulla can be appreciated on the dorsal surface of left little finger.

Another extensive study was done by Experts at Advancer. Among the 113 cases recruited in their study, 37.16% cases had trophic ulcers and 33.62% cases had auto-amputation of digits [12], (Figures 6-10).

Figure 6: B/L complete claw hand with absorption of fingers seen.

Figure 7: B/L complete claw hand with unilateral left upper limb lymphedema.

Figure 8: Photograph of right foot twisted toes and absorption of toes.

Figure 9: Photographs of trophic ulcers seen on the feet.

Figure 10: Photograph of a case having right below knee amputation along with mutilating deformity of the left leg.

Existing literature suggests that claw hands, trophic ulcers and absorption of digits are the most commonly encountered deformities of hands and feet in cases of Leprosy. Keen and deliberate efforts have to be undertaken to assure the rehabilitation.

Conclusion

We intend to prompt, that Leprosy and its related deformities is still a deeply rooted problem in our society with substantial impact on social and personal lives of patients. The deformities of hands and feet are by far the most testing for patients. Constant determination to eliminate Leprosy, keen motivation and dynamic future strategies have to be adopted for betterment. It is also pertinent to make note, that after the advent of MDT, occurrence of deformities has been on a steady low. But, those unfortunate with existing mutilations have to be cared for by bringing them in vogue with measures of self-help and rehabilitation. Persistent counselling should be provided to maintain their morale.

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