I recently encountered an interesting medical case one might have expected to encounter more in the Middle Ages than on a busy morning clinic in Co Meath. 

CC is a 37-year-old Brazilian national who has been living and working in Ireland for the past seven years. His presenting complaint was a two month history of progressively worsening shooting pains of the hands and feet, along with an itchy rash which had started on his torso and spread to his arms and legs. I initially found it difficult to tie the two complaints together and it is safe to say my list of differential diagnoses was limited. 

History-taking and examination

On examination, the rash consisted of diffuse erythematous patches which were macular in nature, patchy, and spread predominantly around the lower abdomen and trunk. A neurological examination showed a painful sensory loss in a glove and stocking distribution. 

On questioning if he had ever experienced anything like this before, he revealed that he had been treated for a similar problem 10 years earlier while living in Brazil. Unsure of the English term for the condition, he wrote ‘Hanseníase’ on a sheet of paper. I was surprised when I discovered the word directly translated to ‘Hansen’s disease’ or ‘leprosy’. Leprosy has a long incubation period of up to seven years. On reviewing the diagnosis it seemed reasonable to pursue further investigations. A referral was made to the infectious diseases service in St James’s Hospital, Dublin, and the patient was reviewed there two weeks later.

History-taking and examination were performed and skin biopsies were obtained from affected areas. These biopsies revealed scattered dermal non-necrotising granulomas, including perineural granulomas. The report suggested the findings were suggestive of tuberculous leprosy or sarcoidosis. No micro-organisms were seen on Gram or Ziehl-Neelsen stains. These findings, together with the clinical history, supported a diagnosis of tuberculoid leprosy.

Features of leprosy

Leprosy is a chronic inflammatory disease caused by the bacillus Mycobacterium leprae. It is primarily a granulomatous disease of the peripheral nerves and mucosa of the upper respiratory tract; skin lesions are the primary external sign.¹ Over time, if left untreated, it will cause permanent damage to the skin, nerves, limbs and eyes. These effects are largely due to loss of sensation which can lead to undetected trauma and secondary infection, resulting in the characteristic limb and facial deformity in severe forms of the condition.

The disease has affected humanity for over 4,000 years,² and was recognised in the civilisations of ancient China, Egypt and India. WHO figures show approximately 219,000 new cases were reported in 2011, with the highest prevalence in countries such as India and Brazil.³ It is uncommon in Ireland, where it is a notifiable disease. The risk of contagion in new cases is small;⁴ however, global eradication in recent years has proven difficult due to high levels of multi drug-resistant bacilli, as well as a poor understanding of the disease and persistent social stigma in developing countries. The forced isolation of patients is unnecessary in places where adequate treatments are available. However, many leper colonies remain around the world in countries such as India.⁵

It is believed that approximately 95% of people have a natural immunity to leprosy due to genetic differences in cell-mediated immunity, and men are twice as susceptible as women.⁴ There are two clinical variants. People with an ineffective immune response are likely to develop the lepromatous form of the disease. People with a functional immune system tend to develop milder tuberculoid disease.

The treatment of leprosy may involve surgery, chemotherapy and physiotherapy. An Irish physician, Dr Vincent Barry (1908-75), is credited with the discovery of the drug clofazimine which makes up part of a multidrug regimen used worldwide today.⁶

This may be an extraordinary consultation to present as a case report from Irish general practice. It is, however, an interesting example of the sort of tropical illnesses that can occur in an ever-changing multicultural Ireland. As we open ourselves to global travellers we can expect to see more global health complaints.  

Acknowledgement: GP Trainer, Marie Scully 

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