Leprosy

Dr. Sherif Diaa Specialist Dermatologist

Definition
Chronic infectious disease of man, caused by Mycobacterium Leprae, affecting peripheral nerves, skin and sometimes other tissues.

Mycobacterium Leprae

Enormous numbers of acid fast bacilli, singly or in clumps.

3

Epidemiology
 

 

The rate at which leprosy spreads in a community depends on the proportion of susceptible individuals in the population The incidence of conjugal leprosy (leprosy acquired from marriage partner) confirms that adults are relatively nonnonsusceptible, for only 5% of those at risk acquire the disease this way. when one parent has the infectious form of the disease and remains untreated, up to 60% of the offspring may develop leprosy as children or young adults. Serological studies confirm that in endemic countries most children get infected. Most infections remain asymptomatic. infected. It tends to have bimodal age distribution, with peaks at 10-14 10and 35-44 years. 35-

Epidemiology
 

The incubation period is usually 3-5 years. 3Nasal discharges from individuals with lepromatous leprosy are thought to be the main source of the infection in the community. Intact skin of lepromatous patients sheds relatively few organisms, but shared clothes and sleeping mats provide important means of transdermal infection.
5

Clinical picture
Early lesions  The commonest early lesion is an area of numbness on the skin or a visible skin lesion, commonly found on the face, extensor surface of limbs, buttocks, or trunk. It consists of one or few slightly hypopigmented or erythematous macules, a few centimeters macules, in diameter, with poorly defined margins. Hair growth and nerve function are unimpaired.  Nasal stuffiness, discharge, or epistaxis are symptoms of early lepromatous leprosy.
6

Clinical picture

Early lesion
7

Clinical picture
Tuberculoid leprosy  The only tissues showing clinical evidence of involvement are nerves and skin. The skin. lesions are few, often solitary. The condition few, may be purely neural, with pain and swelling of the affected nerve followed by anesthesia and/or muscle weakness and wasting.

Clinical picture


The typical lesion is a plaque which is conspicuous, erythematous, copper coloured or purple, with raised and clearclear-cut edges from which there is gradual slope towards a flattened and hypopigmented center. The surface is dry, hairless and insensitive, and sometime scaly.
9

Tuberculoid leprosy

solitary, anesthetic, and annular lesion of tuberculoid leprosy

10

Untreated tuberculoid leprosy, associated peripheral neuropathy



60-year60-year-old woman began to develop ulcers on her hands and feet 15 years earlier Lesions healed with fibrosis and resorption of distal phalanges
11

Clinical picture
Lepromatous leprosy  The most important symptoms are nasal symptoms such as stuffiness, discharge and epistaxis. Oedema of the legs and ankles due to increased capillary stasis and permeability.

12

Clinical picture
 

Dermal signs consist of macules, diffuse papules, infiltration or nodules, or all four. Macules are small, multiple, erythematous or faintly hypopigmented, with vague edges and shiny surface. Papules and nodules are usually of normal skin color, bilateral, symmetrical, and favor face, arms, legs and buttocks. But not hairy scalp, axillae, groins and perineum regions with highest temperature.
13

Lepromatous leprosy

Papules and nodules

14

Lepromatous leprosy

Loss of eye brows, plaques and infilteration on the face

15

Lepromatous leprosy

Lepromatous leprosy Multiple, skin-colored, papular and small nodular lesions

16

Clinical picture


Hair growth and sensation are not at first impaired over the lesions. The longest peripheral nerve fibers that supply sensation are first affected, causing numbness and anesthesia on dorsal surfaces of hands and feet, and later on extensor surfaces of arms and legs, and finely over the trunk.
17

Clinical picture


Infiltration of corneal nerves causes anaethesia, which predisposes to injury, infection and blindness if there is lagophthalmos due to damage to facial nerve. The hands and feet swell. Radiographs show osteoporosis in the phalanges, small osteolytic cysts. Fingers may become crooked or short. Nails are thin and brittle
18

lagophtalmus

19

Clinical picture
If the patient remains untreated  lines of the forehead become deeper as the skin thickens (leonine faces).  Eyebrows and eyelashes become thinned or lost (madarosis).  Ear lobes are thickened.  Nose becomes misshapen and later collapse due septal perforation and loss of anterior nasal spine.  Voice become hoarse, the upper incisor teeth loosen or fall out.  skin of the legs become ichthyotic and thickened, nodules may break down to form ulcers.
20

Lepromatous leprosy

Eyebrows and eyelashes become thinned or lost (madarosis) nose becomes misshapen and later collapse due septal perforation and loss of anterior nasal spine.
21

multiple stigmata of old lepromatous leprosy

 

absorption of digits destruction of the nasal cartilage loss of eye brows

22

Clinical picture


Slow fibrosis of peripheral nerves results in nerve thickening and bilateral anaethesia. Sensation of palms and soles is retained until late in the disease. Leprous deposits in the eyes cause keratitis, iridocyclitis and iris atrophy. Testicular atrophy causes sterility, impotence and gynaecomastia. The commonest cause of death is renal failure; also pulmonary tuberculosis is a common cause. Rarely death may occur from a sever reaction state.

23

Clinical picture
Tuberculoid Number of lesions Distribution Definition and clarity 1-10 Asymmetrical, anywhere Define edge, markedly hypopigmented Early, marked, defined, localized to skin lesions or major peripheral nerve Early in skin and nerve lesions Marked, in a few nerves Absent Not detectable Lepromatous Hunderds, confluent Symmetrical, avoiding spared areas Vague edge, slight hypopigmented Late, initially slight, ill defined, but extensive Late, extensive Slight but widespread Common Numerous in all affected tissue
24

Anaethesia Autonomic loss Nerve enlargement Mucosal and systemic Number of M. leprae

Clinical picture
Borderline leprosy  Skin lesions are intermediate in number between those of the two polar types. The lesions are distributed asymmetrically. They may take the form of macules, plaques, macules, plaques, annular lesions or bizarre-shaped bands. bizarrebands.  Towards the tuberculoid end the lesions are fewer, drier, with more hair loss, more anhidrosis, anhidrosis, more insensitive, with fewer bacilli in smears. And vice versa towards the lepromatous pole.
25

Clinical picture


One or more nerves are likely to be thickened and non-functioning, and neural nonsymptoms may precede the appearance of skin lesions, by as much as 8 years. Borderline disease is the commonest type of leprosy encountered. Borderline disease unstable and tends to downgrade towards lepromatous especially if untreated or upgrade towards tuberculoid.
26

borderline tuberculoid

incompletely annular configuration with satellite papules. less erythema, no evident scales, sharp margination.

27

borderline tuberculoid leprosy

Hypopigmented, slightly erythematous, well defined plaques

28

borderline tuberculoid leprosy

Large, well defined, hypopigmented plaques

29

Borderline leprosy

multiple hypopigmented anesthetic patches scattered over the body 30 Many peripheral nerves were thickened and tender

borderline tuberculoid leprosy



Recurrent reactions in this untreated patient led to paralysis of his right hand resulting in clawing and atrophy of hand muscles. Trophic ulcers and gangrene of the middle and ring fingers required amputation.
31

borderline tuberculoid leprosy

well defined trophic ulcer with a hyperkeratotic border and a moist, beefy-red, granular base on insensitive left sole

32

borderline lepromatous
 

annular lesions asymmetrically distributed Loss of sensation

33

borderline lepromatous leprosy



  

multiple, varying-sized, varyinground and oval, annular, erythematous, indurated plaques Distributed symmetrically asymptomatic no nerve thickening and the lesions were sensitive to touch and pain
34

Diagnosis


Slit skin smear

With small bladed scalpel an incision is made 5 mm long and 3 mm deep, and then the wound is scraped several times in one direction, the fluid from the dermis collect on one side of the blade and then gently smeared on to a glass slide. The smear is stained with modified Ziel-Nielsen Zielmethod. Lepromatous and most borderline leprosy lesions are positive for acid fast bacilli. Under standard treatment, bacilli disappear from borderline lesions in few months, but it may take 6-10 years for the last bacillary remnants to disappear from skin in lepromatous leprosy. The last positive site is dorsa of fingers.
35

Diagnosis


 

Nasal scrape This is useful to establish whether a patient is potentially infectious. Smear will be positive only in lepromatous leprosy. Skin biopsy Nerve biopsy In pure neural tuberculoid or borderline leprosy it will be necessary to remove a small portion of a thickened peripheral nerve in order to establish the diagnosis, so long as the nerve is purely sensory, e.g. great auricular nerve in the neck, radial cutaneous nerve at the wrist, superficial peroneal nerve in front of the ankle, or sural nerve below lateral malleolus.
36

Treatment


1. 2.

Treatment of paucibacillary leprosy Patient whose slit skin smears are ve or who are classified as indeterminate, tuberculoid leprosy. Adult patients are treated as follow Rifampicin 600mg once-monthly oncedapsone 100mg once-daily. onceTreatment is given for 6 months.
37

Treatment
Treatment of multibacillary leprosy This includes all patients in who slit skin smears are +ve, or who are diagnosed as Lepromatous or Borderline leprosy. Adult patients treated as follow,


1. 2. 3. 4.

Rifampicin 600mg once-monthly 600mg onceDapsone 100mg daily 100mg Clofazimine 50mg daily 50mg Clofazimine 300mg weekly 300mg

Treatment is given for 2 years or for 3 years if treatment was irregular. WHO recommends continuing treatment until smear negativity .
38