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Leprosy

Leprosy is characterized as a chronic infectious disease that may cause severe and disfiguring skin sores and neuropathy to the extremities. Because this disease is shrouded in terrifying stigmata in ancient times, lepers are often times out casted from the society. Any outbreaks of leprosy in any country are now met with substantial panic due to this negative stigmata in our past.

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Presentation

The main symptomatology in leprosy are the cutaneous pale sores and lumps which typically persist from weeks to months. In the same way, significant nerve damage leads to peripheral neuropathy that may lead to paresthesia or loss of sensation in arms and legs. Bacillary invasion of the eyes may occur and cause corneal ulcerations and iridocyclitis of the affected eyes [8]. There will also be a consequent paralysis presenting as muscle weakness in the extremities. The symptoms will surface usually after 3 to 5 years from initial contact with an infected carrier.

Entire Body System

  • Fever

    Here we report a case in which an elderly female presented with malar rash, intermittent fever, and arthralgia. Her diagnosis was significantly delayed due to a close clinical resemblance to systemic lupus erythematosus. [ncbi.nlm.nih.gov]

    ‘Programmes to control leprosy, hepatitis and dengue fever are vividly shown.’ ‘In 1879, Albert Neisser, one of Robert Koch's pupils, visited Bergen to study leprosy.’ [en.oxforddictionaries.com]

  • Amyloidosis

    Here, we present the case of a patient with nephrotic syndrome caused by secondary amyloidosis, chronic peripheral neuropathy and a history of leprosy. [ncbi.nlm.nih.gov]

    Hepatic amyloidosis may occur, while pancytopenia due to infiltration of bone marrow, bacteremia, and lymphadenopathy are often seen. [news-medical.net]

    Kidneys are spared unless immune complex nephritis or amyloidosis occurs. [atsu.edu]

    Secondary amyloidosis. Secondary amyloidosis leading to renal impairment may occur when reactions are not well controlled. Neuritis and permananet nerve damage. [clinicaladvisor.com]

  • Gangrene

    Gangrene may also follow, causing body tissue to die and become deformed. [medical-dictionary.thefreedictionary.com]

Respiratoric

  • Saddle Nose

    BACKGROUND: Facial deformation as a sequela of leprosy is caused not only by a saddle nose but also by regression of the maxilla, as well documented in paleopathological observations of excavated skeletal remains of patients with leprosy. [ncbi.nlm.nih.gov]

    […] symptoms and signs 3,6: cutaneous erythematous plaques peripheral lower limb edema mixed peripheral neuropathy (often with resultant injuries) +/- hypoesthesia/paresthesia +/- neuralgia peripheral nerve thickening, which may be palpable corneal ulcers saddle-nose [radiopaedia.org]

    The bone involvement may also lead to gross deformation of the nose skeleton and ensuing creation of a saddle-nose deformity. [news-medical.net]

    In an advanced stage, the symptoms are similar to those observed in LL, with nasal obstruction and epistaxis followed finally by the ulceration of the septum, leading to a deformed ‘saddle-nose’ appearance. [internationaltextbookofleprosy.org]

Gastrointestinal

  • Choking

    2nd riff for "Choke on It" was originally used in a rare "Rigor Mortis" song, which was released as a demo in 1985. US version comes in gatefold cover with lyrics and band photos on the inside. Canadian version comes in non-gatefold cover. [metal-archives.com]

Eyes

  • Band-Shaped Keratopathy

    intersticial keratitis, band shaped keratopathy, corneal leproma. [eyewiki.aao.org]

Skin

  • Papule

    A 42 year old male presented with multiple, discrete, hyperpigmented, firm, non elastic, non tender papules and plaques on the posterior trunk of 5 months duration, resembling keloid. [ncbi.nlm.nih.gov]

    A variety of skin lesions may be seen but macules (flat), papules (raised), or nodules are common. Sensory loss is a typical feature of leprosy. The skin lesion may show loss of sensation to pin pick and/or light touch. [who.int]

  • Eczema

    Types & treatments Childhood eczema Adult eczema Insider secrets Types of hair loss Treatment for hair loss Causes of hair loss Hair care matters Insider secrets What is psoriasis Diagnosis & treatment Skin, hair & nail care Triggers Insider secrets What [aad.org]

    Both men noticed changes in the skin colouring and sensation but GPs initially thought it was due to skin infections or a form of eczema. [telegraph.co.uk]

    For those with skin conditions, including eczema & psoriasis, physical contact with others can be missing in their lives. [skinema.com]

    The skin patch is not eczema, which my own 10-year-old daughter suffers from, but leprosy. The disease causes nerve damage and creates what's called "anaesthetic skin". [news.bbc.co.uk]

  • Skin Ulcer

    He also had Lucio's phenomenon, characterized by vascular thrombosis and invasion of blood vessel walls by leprosy bacilli, causing extensive skin ulcers. [ncbi.nlm.nih.gov]

    ulcerations due to massive AFB burden in internal organs.[1] M. lepromatosis, like M. leprae, has not been cultured in the laboratory because they both lack genes necessary to grow outside their hosts. [en.wikipedia.org]

    It primarily affects the nerves of the extremities, the skin, the lining of the nose, and the upper respiratory tract. Leprosy is also known as Hansen’s disease. Leprosy produces skin ulcers, nerve damage, and muscle weakness. [healthline.com]

    All participants had skin damage at trial entry. Typical outcome measures were: number of ulcers healed; size of ulcers or reduction of ulcer size; number of new ulcers; ulcer free at one year; ulcers not healed; ulcers fully healed. [doi.org]

  • Anhidrosis

    Skin denervation leads to anhidrosis and poor autonomic function, as a result of which ulceration and secondary infection occurs. This progressively erodes the fingers and leaves only stumps behind. [news-medical.net]

    Features which may be seen in leprosy include: Skin lesions - hypopigmented macules and plaques (common), papules and nodules (rare) Neurological involvement - damage to small nerves to the skin, producing reduced sensation and anhidrosis, or peripheral [patient.info]

    The sweat test demonstrates anhidrosis and the complete triple response is not seen in the histamine test [13]. The prognosis is good, as a TT lesion can even self-heal. However, nerve damage-related disability can occur. [internationaltextbookofleprosy.org]

    Abnormalities of autonomic nervous system Autonomic nerve involvement is manifest clinically as varying degrees of impaired sweating and possible anhidrosis. [4] In general, visceral autonomic nerves are not involved, though conflicting experiences with [emedicine.com]

  • Alopecia

    Grossly, all these squirrels presented with bilateral areas of variable alopecia and cutaneous swelling of the snout area, lips, eyelids, pinnae and the distal aspect of all limbs (Fig 1). [doi.org]

    Independent Minds subscription for just £5.99 €6.99 $9.99 a month Get the best of The Independent Without the ads – for just £5.99 €6.99 $9.99 a month The disease seems to cause similar symptoms in red squirrels, with individuals commonly exhibiting alopecia [independent.co.uk]

    Occasionally, the scalp may be involved, in which case the hair loss may mimic alopecia areata. Nasal mucosa is involved insidiously with an initial congestive phase in which the mucosa is swollen with numerous telangiectasia. [internationaltextbookofleprosy.org]

Psychiatrical

  • Social Isolation

    It's caused by a long-term bacterial infection, and though it was once viewed as a highly contagious disease that led to social isolation, today it is considered rare and easy to treat, according to the Centers for Disease Control and Prevention. [ibtimes.com]

    For centuries, leprosy remained a poorly understood disease characterized by human suffering and social isolation. In 1873, G.A. Hansen discovered the bacterial cause of this infectious disease. [emedicinehealth.com]

    Such stigma often remains long after a person has been cured.[55] This can lead to social isolation, depression, and even suicide. [en.wikipedia.org]

Neurologic

  • Peripheral Neuropathy

    Here, we present the case of a patient with nephrotic syndrome caused by secondary amyloidosis, chronic peripheral neuropathy and a history of leprosy. [ncbi.nlm.nih.gov]

    In the same way, significant nerve damage leads to peripheral neuropathy that may lead to paresthesia or loss of sensation in arms and legs. [symptoma.com]

  • Tingling

    Some may experience muscle weakness, numbness and/or tingling in a body part, or upper respiratory problems (including a runny nose, eye, and ear problems). Combinations of the above symptoms may also occur. [aocd.org]

    In particular, leprosy patients are susceptible to certain complications: [14] Neuritis, silent neuropathies (nerve damage without pain), pain, burning, tingling, and sudden numbness may occur. This can be treated with corticosteroids. [wikihow.com]

    The symptoms of nerve involvement include diminished sensation or feeling in the affected areas (anesthesia) and, sometimes, burning and tingling sensations (paresthesias). [rarediseases.org]

    Unexplained skin lesions or rash Loss of sensation or tingling of the skin Thickening of the skin Muscle weakness and/or numbness in the extremities Eye pain or vision changes It is important to note that the following findings may not be apparent for [emedicinehealth.com]

  • Paresthesia

    An 18-year-old man presented with a 4-year history of erythematous patches on the trunk, followed 2-years later by multiple nodules, mostly located on the limbs, and distal paresthesias. [ncbi.nlm.nih.gov]

    […] period of ~3 years (range: 6 months-20 years) between infection and onset of multi-systemic symptoms and signs 3,6: cutaneous erythematous plaques peripheral lower limb edema mixed peripheral neuropathy (often with resultant injuries) +/- hypoesthesia/paresthesia [radiopaedia.org]

    In the same way, significant nerve damage leads to peripheral neuropathy that may lead to paresthesia or loss of sensation in arms and legs. [symptoma.com]

    […] branches, resulting in inability to close eye (lagophthalmos) Trigeminal Emerging from respective foramina Anesthesia of face, cornea, & conjunctiva Supraorbital Above the eyes Cosmetic importance only *Early manifestations of leprosy may include local paresthesias [atsu.edu]

    Scott Smith, MD) Loss of sensation or paresthesias where the affected peripheral nerves are distributed Wasting and muscle weakness Foot drop or clawed hands (may result from neuritic pain and rapid peripheral nerve damage; as seen in the image below) [web.archive.org]

  • Hypesthesia

    The hallmark signs of leprosy are hypesthesia (an abnormally weak sense of pain, cold, heat, or touch), skin lesions, and peripheral neuropathy. The first indications someone has leprosy are usually have to do with the skin. [todayifoundout.com]

  • Burning Sensation

    A case of Kilásam. due to the action of the deranged Pittam. is marked by eruptions, resembling the petals of a lotus flower (in shape and colour), and are attended with an extremely burning sensation. [chestofbooks.com]

Workup

The characteristic cutaneous lesions with history of exposure to an infected person may give the primary physician a clue that he is dealing with a leprosy case. To further confirm the diagnosis, the following diagnostic modalities and tests may be performed to patients presenting with signs and symptoms of leprosy:

  • Skin biopsy or slit skin test will demonstrate the actual bacteria after an acid fast staining technique is applied overt the sample skin tissues under microscopy [9].
  • Skin scrapings examination in which the surface of the cutaneous lesions are scraped using a scalpel and stained with acid fast demonstrate the Mycobacterium leprae organisms.
  • Lepromin test distinguishes lepromatous from tuberculoid type of leprosy.

Treatment

The following anti leprosy medications or antibiotics are commonly used in the treatment of both forms of leprosy:

  • Rifampicin
  • Dapsone
  • Clofazamine
  • Fluoroquinolones
  • Minocycline
  • Erythromycin and other macrolides

Medications may also be given to control the inflammations associated with leprosy, these include:

  • Aspirin
  • Thalidomide 
  • Prednisone [10]

Prognosis

Leprosy as a disease is rarely fatal, complications of nerve damage predisposes patients to other morbid events. Early diagnosis and early treatment are paramount in all cases of leprosy. This will limit nerve damage and disability, limit the spread of the disease, and increase the chances of the patient to live a normal life.

Etiology

Leprosy is caused by the acid fast mycobacterium leprae which is not very contagious. The incubation period of M. leprae may take months to years making it almost impossible to pinpoint with certainty where the disease was initially contracted. Children are more likely to contract the disorder compared to their adult counterparts. Leprosy occurs in two forms; the tuberculoid and the lepromatous form.

Both forms will present with skin sores and neuropathies, but the lepromatous forms are more severe in terms of cutaneous presentation. Leprosy is still common in tropical, subtropical, temperate countries. The emergence of the multidrug resistant strains of Mycobacterium leprae have raised global concerns regarding leprosy worldwide [2].

Epidemiology

In the United States, around 205 new cases of leprosy were reported by the Registry of the National Hansen’s Disease Programs as of 2010 [3]. Although majority of this cases are diagnosed among immigrants from foreign countries like Asia, Africa and Latin America. Anthrozoonotic transmission of leprosy has been noted from infected armadillos in the southern part of the United States [4]. Internationally, there has been a significant decline in the prevalence rate of leprosy as of 2010 as compared to baseline data from 2004. In one of the recent prospective studies, newly diagnosed cases of leprosy may exhibit signs of impaired nerve function in up to 56% [5].

Leprosy is still considered to be the most common cause of hand crippling especially those involving the ulnar nerve [6]. There is no racial predilection for leprosy worldwide. There is a 3:2 male to female ratio among leprosy infected patients globally, but in some areas of Africa women are more prone to the disease than men [7]. Age specific incidence of leprosy has a mean age of 10 years old representing almost 20% of all the leprosy cases.

Pathophysiology

The clinical manifestation of leprosy differs significantly according to the cellular response of the infected host. When the host possess a hyperactive cellular response, patients tend to have the tuberculoid form or the paucibacillary form of leprosy characterized by dry cutaneous lesion and hypoesthetic nerves. Skin test and slit test will not reveal any actual bacteria on microscopy but the leprae antigen will test positive in this case. Patients having minimal response exhibit the lepromatous form or the multibacillary form of the disease which is characterized by extensive and severe skin involvement and symmetric nerve dysfunctions. Skin test will herald a positive results of the mycobacterium.

Prevention

The ultimate approach to prevention is to avoid direct close contact with untreated leprosy patients. Patients should submit to immediate treatment after confirmatory diagnosis to reduce the risk of permanent disability and physical disfigurement.

Summary

Leprosy or Hansen’s disease is a chronic infection brought about by the acid fast rod bacilli Mycobacterium leprae. Leprosy is a disease that causes damage to both skin (acutely) and nerves (latent infection) which can manifest from several months to years from exposure.

The World Health Organization (WHO) has launched a global initiative to eradicate the disease and bring down the prevalence rate to less than 1 in 10,000 population in the year 2000. This campaign was successfully met in 2002, save for the 15 out of 122 countries that were previously endemic as of 1985 [1]. Multidrug regimens were used worldwide to treat more than 14 million leprosy patients since 1985. Access to the multidrug medications is still the leading problem in the global leprosy campaign especially in some endemic regions of the world. The WHO is bent on reducing the worldwide incidence rate by 35% by 2015.

Patient Information

Definition

Leprosy or Hansen’s disease is a chronic infection brought about by the acid fast rod bacilli Mycobacterium leprae.

Cause

Leprosy is caused by the acid fast Mycobacterium leprae.

Symptoms

Skin bumps and lumps with hypoesthesia and peripheral neuropathies are common symptoms.

Diagnosis

Diagnosis is done by a detailed clinical history and direct physical examination of the lesions. Confirmatory tests including skin biopsy, skin scraping tests, and lepromin tests may be implored.

Treatment and follow-up

Anti-leprosy medications like rifampicin, dapsone and clofazimine. Anti-inflammatory agents like aspirin, thalidomide, and prednisone may also be used to allay the symptoms.

References

  1. World Health Organization. Global leprosy situation, 2005. Wkly Epidemiol Rec. Aug 26 2005; 80(34):289-95.
  2. Joyce MP, Scollard DM. Leprosy (Hansen's Disease). Conn's Current Therapy. 2004; 100-105.
  3. Truman RW, Singh P, Sharma R, et al. Probable zoonotic leprosy in the southern United States. N Engl J Med. Apr 28 2011; 364(17):1626-33.
  4. Ustianowski AP, Lockwood DN. Leprosy: current diagnostic and treatment approaches. Curr Opin Infect Dis. Oct 2003; 16(5):421-7.
  5. Anderson GA. The surgical management of deformities of the hand in leprosy. J Bone Joint Surg Br. Mar 2006; 88(3):290-4.
  6. Scollard DM, Adams LB, Gillis TP, Krahenbuhl JL, Truman RW, Williams DL. The continuing challenges of leprosy. Clin Microbiol Rev. Apr 2006; 19(2):338-81.
  7. Walker SL, Lockwood DN. Leprosy. Clin Dermatol. Mar-Apr 2007; 25(2):165-72.
  8. Anderson H, Stryjewska B, Boyanton BL, et al. Hansen disease in the United States in the 21st century: a review of the literature. Arch Pathol Lab Med. Jun 2007; 131(6):982-6.
  9. Van Veen NH, Nicholls PG, Smith WC, Richardus JH. Corticosteroids for treating nerve damage in leprosy. Cochrane Database Syst Rev. Apr 18 2007; CD005491.
  10. Kai M, Nguyen Phuc NH, et al. Analysis of Drug-Resistant Strains of Mycobacterium leprae in an Endemic Area of Vietnam. Clin Infect Dis. Mar 2011; 52(5):e127-32.
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