QuestionSBA
QuestionChoice
Daktacort is hydrocortisone with miconazole and is used mostly in mildly inflamed skin disorders with associated infection. It is not appropriate in this case due to the history being suggestive of leprosy
32814
b
Daktacort (daily)
QuestionChoice
Rifampin, isoniazid, pyrazinamide, ethambutol are the regimen for the treatment of tuberculosis. Cutaneous tuberculosis is rare and is due to direct inoculation with tubercle bacilli into the skin. It does not generally lead to paraesthesia. Other types of mycobacterial skin infections include leprosy, which is what the patient is suffering from in this case
32817
e
Rifampin, isoniazid, pyrazinamide, ethambutol (all daily)
QuestionChoice
Betnovate is a potent topical corticosteroid. It is often prescribed in severe inflammatory conditions. It is not appropriate in this case due to the history being suggestive of leprosy
32815
c
Betnovate (as required)
QuestionChoice
Metformin is the first line treatment of type II diabetes mellitus. The patient is displaying unilateral paraesthesia symptoms, which are less likely to be due to a peripheral nephropathy secondary to diabetes. The muscle wasting is a sign of peripheral nerve thickening, which suggests leprosy
32816
d
Metformin (daily)
QuestionChoice
This patient has presented with features of leprosy. Therefore, the best management would be for multidrug therapy. The gold standard for diagnosis would be to await a skin swab which showed Mycobacterium leprae. A positive swab plus hypopigmented skin lesions with reduced sensations and peripheral nerve thickening are the three features required to make a leprosy diagnosis. This patient is displaying signs of left ulnar nerve thickening
32813
a
Rifampicin (monthly), dapsone (daily)
QuestionComment
How do you know what left ulnar thickening is?

7920
User
subscriber
Acute Myotonia
QuestionComment
harsh
43294
QuestionComment
spend more time on the wards :/
45907
User
subscriber
Myotonia Gland
User
subscriber
Kinase Hallux
Concept
Chapter
# Summary
 
 
Leprosy is a disease endemic in many developing countries. The disease can manifest in various ways depending on host immunology and bacterial virulence. The two main forms are multibacillary (lepromatous) and paucibacillary (tuberculoid) leprosy. Differential diagnoses to consider include inherited diseases, endocrine disorders, and other conditions such as AL amyloidosis. Diagnosis is primarily based on clinical assessment and the presence of acid-fast bacilli in biopsies or smears. Management involves the use of medications such as dapsone, rifampicin, and clofazimine, with close monitoring for potential side effects and immunological complications. 
 
 
# Definition
 
 
Leprosy is a mycobacterial disease caused by *Mycobacterium leprae* which typically presents with dermatological and neurological manifestations.
 
# Epidemiology
 
Leprosy is endemic in a number of developing countries across the world. Most new cases occur in Southeast Asia.
 
It is associated with severe morbidity, reduced psychosocial functioning and stigmatisation. While difficult to transmit, it is thought to be spread by the respiratory route via nasal discharge.
 
# Aetiology
 
 - Leprosy is spread through droplets from the respiratory tract. Prolonged close contact (over months) is required to transmit the disease.
 - Leprosy manifests in a number of different ways owing to a range of factors, the most important being host immunology and bacterial virulence/initial infectious load
 - *M. leprae* grows best at 27–33° so it prefers to grow at **cooler** areas of the body (eg. skin, nerves close to the skin, mucous membranes)
 - There are five types, but broadly it can be classified as lepromatous and tuberculoid leprosy
 - In disseminated lepromatous/multibacillary leprosy, bacteria become widely disseminated due to poor Th1 cell-mediated responses. This causes symmetrical peripheral nerve damage through demyelination of peripheral nerves, as well as classical skin changes.
 - In tuberculoid/paucibacillary leprosy, there is a robust Th1 cell-mediated response, leading to better control by the immune system and milder clinical manifestations.
 
# Symptoms & Signs
 
The clinical features of leprosy exist on a spectrum. At one end is **disseminated lepromatous/multibacillary leprosy**:
 
 - Coppery or hypopigmented anaesthetic patches; ≥5
 - Classic facial changes include nose destruction and ear swellings – leonine faces
 - Nerve thickening may be felt on palpation, with the most commonly affected nerves being the ulnar, median, radial cutaneous, greater auricular, common peroneal and posterior tibial nerves to control of the infection
 
Clinical features of **tuberculoid/paucibacillary leprosy** include a milder form of nerve damage and dermatological manifestations (<5 lesions)
 
Nerve damage in all forms can lead to contractures, ulceration and deformity in the long term
 
# Differential diagnosis
 
 
The differential diagnosis for other causes of thickened peripheral nerves include:
 
 - **Inherited diseases** – Charcot–Marie–Tooth disease, Refsum's disease and neurofibromatosis. These can also cause peripheral neuropathies and cutaneous manifestations alongside a family history.
 - **Acromegaly** - coarsening features & nerve compression (especially carpal tunnel syndrome). Symptoms are more generalised and complications such as diabetes mellitus can develop.
 - **AL amyloidosis** - may be associated with symptoms of multiple myeloma (hypercalcaemia, renal dysfunction, anaemia, bone pain).
 - **Peripheral neuropathy** secondary to diabetes mellitus, alcohol use or, more acutely, Guillain-Barré syndrome. This is usually preceded by poorly controlled diabetes or alcoholism, or by a recent infection (precipitating GBS)
 
 
# Diagnosis
 
Leprosy is diagnosed with one or more of the following clinical features and laboratory tests:
 
 - Loss of sensation in a hypopigmented or reddened skin patch
 - Thickened peripheral nerve and sensory loss/weakness in the area supplied by the nerve
 - Slit-skin smear demonstrating acid-fast bacilli with a special stain
 
 
# Treatment
 
Early diagnosis and treatment can reduce illness severity and long-term sequelae.
 
 - Treatment of multibacillary leprosy involves the use of dapsone, rifampicin and clofazimine (an immunosuppressive agent) for at least 12–24 months
 - Patients commenced on medications need to be monitored closely throughout the course of their treatment for immunological complications known as type I and II (erythema nodosum lepromum) reactions, which require hospital inpatient treatment. Treatment should continue with the addition of prednisolone, aspirin or thalidomide under specialist guidance.
 - Side effects of dapsone include methaemoglobinaemia, agranulocytosis, Stevens–Johnson Syndrome and the DRESS syndrome; it can also trigger a haemolytic crisis in G6PD deficiency
 - Clofozamine can cause abnormal skin pigmentation
 - Rifampicin can cause orange secretions and is a cytochrome P450 inducer, hence has several drug interactions
 - Treatment of paucibacillary leprosy involves rifampicin and dapsone for 6 months
 
 
# References 
 
 [BNF: Leprosy treatment summary](https://bnf.nice.org.uk/treatment-summaries/leprosy/#:~:text=Paucibacillary%20leprosy%20should%20be%20treated,is%20sufficient%20to%20treat%20tuberculosis.)
 
 [Click here for the WHO page on leprosy](https://www.who.int/news-room/fact-sheets/detail/leprosy)
 
 
 [Leprosy: review of the epidemiological, clinical, and etiopathogenic aspects - Part 1 - PMC (nih.gov)](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4008049/)
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Picture
Leonine facies.
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Leonine facies leprosy.jpeg
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Hypesthetic patches seen in someone with leprosy.
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Multibacillary leprosy lesion.jpeg
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Leprosy
Topic
58
Infectious Diseases
6563
Leprosy is treated with a combination of antibiotics, typically including rifampicin (monthly) and dapsone (daily), as part of multidrug therapy to effectively reduce the bacterial load and prevent transmission.
A 42-year-old woman attends GP due to reduced sensation over a hypopigmented patch of skin. The symptoms have been getting worse for several months. The area of skin affected is a large plaque around 5cm by 6cm over the left upper arm. After taking a careful history, the GP finds out that the patient lived in Colombia before moving to the United Kingdom 5 years ago. On examination, the GP notes wasting of the hypothenar muscles of the left hand and reduced sensation over the lateral half of the 4th digit and the whole of the 5th digit.

Which of these would be the most appropriate management?
a