Volume 26 Number 3

Key messages

• Early diagnosis of the lesions is vital for timely and proper implementation of treatment. This helps to prevent the sequelae of the disease, which could lead to physical disabilities that have an immeasurable impact on the individuals’ social and personal life.
• Due to human migration movements, more cases of Hansen´s disease are to be expected in European territories, and it is fundamental that clinicians recognise and implement a proper treatment plan in a timely manner.
• In the process of providing care for this population of patients, it is crucial to address their wellbeing and promote quality of life, by providing proper pain management, along with odour and exudate control.

Introduction

Leprosy is a chronic infectious disease caused by the acid-fast bacillus Mycobacterium leprae, primarily affecting the skin and peripheral nerves.^1,2 This disease causes severe disfigurement of the affected individual. It is considered an ancient disease, as it has affected humanity for thousands of years. This disease also has tremendous stigma associated with it.³ In spite of all the efforts, the disease remains a major healthcare problem in many underdeveloped and developing countries, like India and Brazil.³ Transmission occurs through respiratory droplets or close, prolonged contact with infected individuals.¹

Despite being declared by the World Health Organisation (WHO) as ‘eliminated’ as a global public health problem, in the year 2000, approximately 200,000 new cases were reported worldwide in 2017.⁴ The accurate incidence of leprosy is challenging to calculate, as it is extremely low. Moreover, not all cases are accurately recognised on their occurrence. There is evidence of delayed identification of cases. A big-time lapse occurs between the commencement of the symptoms and actual diagnosis.³

Around 80% of the reported global new cases are identified in India, Brazil, and Indonesia. Brazil ranks second in terms of absolute number of cases.³ It is theorised that global distribution correlates with human migration¹ and may spread leprosy to non-endemic areas, such as North America and Europe. According to WHO, Europe had the lowest incidence and prevalence of Hansen´s disease, in 2016.⁴ In Portugal, the prevalence is low, with an average of 2-3 cases per year, posing a public health challenge that complicates accessibility to a likely and rapid diagnosis.

Early diagnosis of the lesions is vital for timely and proper implementation of treatment. This helps to prevent the sequelae of the disease, which could lead to physical disabilities that have an immeasurable impact on the individuals’ social and personal life, adding to the stigma regarding this malady.³ Social stigma and discrimination have played a significant role in the trajectory of this disease, limiting its acceptance and, consequently, adherence to treatment.

The diagnosis of leprosy in a patient who has yet to complete a full course of treatment is established on the manifestation of at least one of the three vital signs; a pale or reddish skin patch with localised loss of sensation; a thickened or enlarged peripheral nerve associated with loss of sensation and/or weakness of the muscles supplied by that nerve; and evidence of acid-fast bacilli in a slit-skin smear. The diagnosis is further supported by histopathological examination of skin biopsy.³

The recommended treatment for leprosy consists of rifampicin, dapsone, clofazimine,² ofloxacin and minocycline. Vaccines are also available. However, relapse may occur after treatment has been stopped and patients must be educated on the signs of relapse to allow proper treatment and reduce severity.⁵

With this case study, the authors aimed to share the difficulties of establishing the correct diagnosis of Hansen´s disease and to raise awareness of this condition, especially in European countries where clinicians are not familiarised with the disease. Due to human migration movements, more cases of leprosy are to be expected in European territories, and it is fundamental that clinicians recognise and implement proper treatment plans in a timely manner.

Case description

A 65-year-old Brazilian woman, residing in Portugal for 15 years, autonomous in daily activities, was admitted to the emergency department on August 3, 2023, due to an unmeasured fever since the previous day, accompanied by progressive oedema and erythema of the right lower limb (Figure 1). A skin infection was presumed, and antibiotic therapy with Amoxicillin and Clavulanic Acid was prescribed, with no indication for hospitalisation. She returned to the emergency department two days later due to the sudden onset of a generalised purpuric rash (Figure 2), more pronounced in the left lower limb, with a probable aetiology of small/medium vessel vasculitis and granulomatous polyangiitis, along with a febrile state of four days. Given the inflammatory/infectious characteristics of the lesions, empirical antibiotic therapy was switched to Clindamycin 300mg (every 8 hours). The patient reported a burning sensation without pruritus, scaling, or pain. She denied chills, asthenia, arthralgia, myalgia, gastrointestinal or genitourinary symptoms, as well as respiratory or cardiovascular complaints. There was no history of recent travel or contact with wild animals. The patient also denied any past medical history, usual medication, smoking habits or harmful substance use. The patient mentioned nasal septum bulging for more than two years, with crusted intranasal lesions and episodes of epistaxis caused by nasal manipulation (saddle nose). In December 2022, she consulted her General Practitioner due to significant weight loss (30kg, corresponding to 66.14lbs) over the year, reporting general malaise and nonspecific discomfort mainly in the lower limbs, with erythematous lesions interpreted as leg ulcers. Her medical history included chronic venous insufficiency (without imaging studies) and chronic anaemia (with unclear aetiology).

 

Figure 1. Indolent appearance of erythematous lesions associated with oedema.

 

Figure 2. Sudden onset of generalised purpuric rash with evidence of hypopigmented areas.

 

On August 5, during her first hospitalisation, the patient was alert, febrile, normotensive, with a tendency towards tachycardia, heart rate between 90 and 110 beats per minute, and rhythmic, regular heart sounds with no audible murmurs. She was eupnoeic in room air. Laboratory results showed hypochromic microcytic anaemia, with haemoglobin at 8.5g/dL, and elevated inflammatory markers: CRP (26.9mg/dL), leucocytosis 119,000, neutrophils 87%, PCT 1.3 ng/ml, ESR 94mm/h, ferritin 3078ng/dL.

During hospitalisation, on August 8, a dermatology consultation was requested, and a skin biopsy was performed on the left thigh for histopathological and microbiological examination, revealing “the presence of multiple thrombi and vasculitis in the adipose tissue lobules, along with a neutrophilic infiltrate. Ziehl-Neelsen and Fite staining revealed numerous acid-fast bacilli, often arranged in ‘globi’.” This finding was decisive for the differential diagnosis of multibacillary Hansen’s disease with a type II immunological reaction (erythema nodosum leprosum), with a probable Lucio phenomenon. Lucio’s phenomenon is characterised by erythematous-violaceous macular and slightly infiltrated plaques on the skin, which subsequently lead to central necrosis and ulceration, affecting mainly the limbs but also the trunk and face in the severe cases, without significant systemic involvement initially. Is a type of reaction usually observed in untreated or inadequately treated diffuse forms of lepromatous leprosy and is a life-threatening medical emergency. Lucio’s phenomenon is seen mostly in untreated patients and rarely reported.⁶

On August 10, corticosteroid therapy with Prednisolone 70mg was initiated, resulting in significant improvement of the lesions, with extensive areas of granulation and revascularisation (Figure 3). With the diagnosis established, the patient began the WHO-recommended therapeutic regimen, consisting of a combination of Rifampicin, Dapsone, and Clofazimine, for a duration of 12 months for multibacillary leprosy.

 

Figure 3. Lesion with extensive areas of granulation.

 

The patient was discharged from the hospital on August 14 and returned to the community care team on August 18 for the first treatment of the lesions, initially present on all four limbs (Figure 4).

 

Figure 4. Initiation of treatments at community care team.

 

Treatment

Since the beginning of treatment, progressive improvement of the lesions has been observed, through a very challenging process. Initially, the lesions were extensive, highly exudative with purulent content, and had a strong fetid odour. They were mostly necrotic, with fibrin plaques and suspicious of biofilm (Figure 5). Treatment was adjusted according to sequential evaluation and assessed needs, using dressings for odour and infection control, debridement, and skin hydration, such as activated charcoal, sodiumcarboxymethyl cellulose with silver, hydrogel or collagenase, and vaseline, along with the application of compression therapy with zinc oxide bandages (Unna Boot), the only system available in the community care facility. The treatment frequency varied between two to three times per week, adjusted not only to the patient’s needs but also to the patient’s constraints, such as transportation to the health unit.

 

Figure 5. Necrotic lesions with necrotic plaques and slough.

 

On May 14, the treatment was changed to Sodium Hypochlorite 0.2% with sea salt 3% irrigation solution due to the extent of the wounds, lack of positive evolution, persistent signs of biofilm, and thickened perilesional edges (Figure 6, the last photo before treatment). From the first treatment with Sodium Hypochlorite 0.2% with sea salt 3%, a significant reduction in odour was observed, which the patient reported as absent after the second application, even without the use of any other odour-controlling dressing. Another notably positive change was the reduction of signs of biofilm, with the appearance of small ‘islands’ of granulation tissue along with the thinning of the wound edges, allowing for selective surgical debridement. It is also noteworthy that the wound was covered with povidone-iodine soaked gauze, following skin hydration and massage of the lower limb, and protected with compression bandages, achieving excellent exudate control and allowing for a reduction in wound size with skin growth from the edges inward.

 

Figure 6. Last photographic record before the application of the irrigation solution
with sodium hypochlorite with sea salt 3% on 14/05/2024.

 

The product (Sodium Hypochlorite 0.2% with sea salt 3%) was chosen based on its biocompatibility and its indication for use in complex chronic wounds requiring odour control, while being compatible with other products and simultaneously improving the wound environment, which is essential for healing.

Discussion

Since leprosy disease is “from other times and places,” it is rare in Portugal and typically imported, leading to initial difficulties in differential diagnosis and the institution of targeted treatment. According to the 2019 World Health Organisation (WHO) guidelines for the diagnosis, treatment, and prevention of Hansen’s disease, the diagnosis remains based on the presence of at least one of the three cardinal signs: Definitive loss of sensation in a hypopigmented or erythematous skin patch; Presence of a thickened or enlarged peripheral nerve with loss of sensation and/or muscle weakness supplied by that nerve; Presence of acid-fast bacilli (AFB) on bacilloscopy.^7,8,9 Thus, despite the characteristic lesion presentation, the diagnosis was conclusive only after biopsy results, and strategies were defined accordingly.

Regarding wound treatment, clinical decisions were based on the presented symptoms and the products available at the health unit, with very limited choices and no possibility of using other techniques, such as vacuum/negative pressure therapy. The product’s indications and expected outcomes were presented before its introduction, and the irrigation solution was chosen accordingly.

During this process, nearly a year into its course, a dermatology consultation was conducted on March 14, 2024, which did not propose any changes to the treatment plan, reinforcing to the patient that she was well cared for and that the results were within expectations.

It seems fair to the authors to conclude that the unilateral approach to wound treatment and the limited range of products available in public health services are hindering factors for more rapid and effective wound treatment.

 

Figure 7. Photographic record after the first application of sodium hypochlorite
with sea salt 3% on 14/05/2024.

 

Figure 8. Photographic record on 17/05/2024 before the application of the solution.

 

Figure 9. Photographic record on 17/05/2024 after the application of the solution.

 

Figure 10. Photographic record on 21/05/2024 before the application of the solution.

 

Figure 11. Photographic record on 21/05/2024 after the application.

 

Figure 12. Last photographic record on 11/06/2024.

 

Conclusion

The aim of this case study is to document the difficulties of establishing a correct diagnosis of leprosy, also known as Hansen´s disease, particularly in a European setting, where it is extremely rare and awareness of such a malady is low. The importance of obtaining an accurate diagnosis for proper treatment plan implementation is cardinal. In the process of providing care for this population of patients, it is crucial to address their wellbeing and promote quality of life, by providing proper pain management, along with odour and exudate control.

Author contributions

Literature review, manuscript writing, and critical revision.

Data confidentiality

The authors declare that they have followed their institution’s protocols regarding the publication of patient data.

Informed consent

Obtained.

Conflicts of interest

The authors declare no conflicts of interest regarding the work presented.

Funding sources

This work did not receive any financial support from public or private entities.

Author(s)

Araújo Sílvia¹, Zacarias Leonor²; Ramos Paulo^3,4, Raposo Maria Eduarda^{5   
1}Health Local Unit Loures/Odivelas, USF Génises, Portugal
²Nursing School of São João de Deus, Portugal
³Health Local Unit Póvoa de Varzim/ Vila do Conde, USF Corino de Andrade, Portugal
⁴Portuguese Wound Care Association, Portugal
⁵Medicine faculty of Porto University, Portugal

*Corresponding author email izabel.aivlis@gmail.com

References

1. Ploemacher T, Faber WR, Menke H, Rutten V, Pieters T. Reservoirs and transmission routes of leprosy ; a systematic review. PLoS Negl Trop Dis. 2020;14(4):e0008276. doi: 10.1371/journal.pntd.0008276
2. Alemu Belachew W, Naafs B. Position statement: LEPROSY: Diagnosis, treatment and follow-up. J Eur Acad Dermatology Venereol. 2019;33(7):1205–1213.
3. Sarode G, Sarode S, Anand R, Patil S, Jafer M, Baeshen H, et al. Epidemiological aspects of leprosy. Dis Mon. 2020;66(7):100899. doi: 10.1016/j.disamonth.2019.100899
4. Maymone MBC, Laughter M, Venkatesh S. Leprosy: Clinical aspects and diagnostic techniques. J Am Dermatology. 2023;83(1):1–14. doi: 10.1016/j.jaad.2019.12.080
5. Mungroo MR, Khan NA, Siddiqui R. Microbial pathogenesis Mycobacterium leprae: pathogenesis, diagnosis and treatment options. Microb Pathog. 2020;149:104475. doi: 10.1016/j.micpath.2020.104475
6. Frade MAC, Coltro PS, Filho FB, Horácio GS, Neto AA, da Silva VZ, et al. Lucio’s phenomenon: A systematic literature review of definition, clinical features, histopathogenesis and management. Indian J Dermatol Venereol Leprol. 2022;88(4):464–477.
7. Ferreira, M., Grijó, C., Paulo, J., Fonseca, M., Neves, Z. An uncommon presentation of an ancient disease. Revista Científica da Ordem dos Médicos. 2024;37(3):215–219.
8. World Health Organization. Guidelines for the diagnosis, treatment, and prevention of leprosy. Geneva: WHO; 2019.
9. Rocha, E., Jesus, G., Neves, C., Sá, D., Araújo, E., Pinheiro, B., Borralho, T., Geraldo, P., Fernandes, B., Campos, H., Crespo, J. Hansen’s disease – a disease on the verge of eradication. Revista da Sociedade Portuguesa de Medicina Interna. 2007;15(2):108–111.