Abstract
Leprosy (Hansen’s disease) is caused by Mycobacterium leprae. It affects the skin and peripheral nerves. Incidence and prevalence are underestimated due to challenges in diagnosis and unfamiliarity with the disease in Canada. Untreated disease can lead to permanent nerve damage with subsequent loss of function. We present a case of Hansen’s disease with delay in diagnosis and treatment. A 31-year-old female recent immigrant from the Philippines presented with a 2-year history of a palpable erythematous rash on her face and arms. She had a diagnosis of cryoglobulinemic vasculitis associated with her chronic hepatitis B. A course of topical corticosteroids did not alleviate symptoms. The rash had a waxing and waning pattern that was felt to mirror her antiviral therapy. Although hepatitis B cryoglobulinemia has been reported in the remote literature, further studies have failed to illustrate that it is a significant cause of cryoglobulinemia. Retrospective analysis of the case identified that anchoring to the initial diagnosis was the reason for delay in diagnosis and treatment.
Key words: Canada, Hansen’s Disease, leprosy, Mycobacterium leprae
Abstract
La lèpre est causée par le Mycobacterium leprae. Elle touche la peau et les nerfs périphériques. Son incidence et sa prévalence sont sous-estimées en raison du diagnostic difficile à poser et de la méconnaissance de cette maladie au Canada. Non traitée, cette maladie peut provoquer des lésions nerveuses permanentes, suivies d’une perte de fonction. Les chercheurs présentent un cas de maladie de Hansen qui a tardé à être diagnostiqué et traité. Une néo-immigrante de 31 ans des Philippines a consulté à cause d’une éruption érythémateuse palpable qu’elle avait depuis deux ans sur le visage et les bras. Elle avait reçu un diagnostic de vascularite cryoglobulinémique associé à une hépatite B chronique. Une corticothérapie topique n’a pas soulagé les symptômes. L’éruption avait un aspect de creux et de bosses qui semblait refléter l’antivirothérapie. Même si la cryoglobulinémie causée par l’hépatite B est signalée dans des publications isolées, d’autres études n’ont pu démontrer de lien important entre ces deux affections. L’analyse rétrospective du cas a établi que l’association des symptômes avec le diagnostic initial explique le retard de diagnostic et de traitement.
Mots-clés : Canada, maladie de Hansen, lèpre, Mycobacterium leprae
Case Presentation
A 31-year-old female from the Philippines who immigrated to Canada in 2015 presented with a 2-year history of a palpable erythematous rash on her face and arms. Past medical history was relevant for e-antigen–positive chronic hepatitis B. The patient was employed as a health care aide and lived with her son. She denied recreational drug use, smoking tobacco, and drinking alcohol.
She had a diagnosis of cryoglobulinemic vasculitis associated with her chronic hepatitis. The rash had a waxing and waning pattern following initiation of antiviral therapy (Tenofovir). A course of topical corticosteroids had not alleviated her symptoms.
The rash was initially located on her face and arms but had later progressed to her legs, 1 year prior to this visit. The skin changes were not pruritic or painful, but she had complained of numbness. This led to a presumed diagnosis of carpal tunnel syndrome, as the numbness was exacerbated by repeated movements at work. Carpal tunnel syndrome was confirmed by demonstration of median nerve neuropathy on nerve conduction studies (NCS)/electromyography (EMG). At that point, she had also noticed an ulcer on her right thumb that she felt was associated with repetitive movements from her occupation.
Review of systems was negative. She had no sick contacts, no animal exposures, and no travel outside of Canada following her immigration.
On physical exam, she appeared well. There were several well demarcated lesions with varying degree of erythema and hypoesthesia spread over her extremities and her back. (Figure 1) The largest lesion was on her right proximal arm measuring approximately 10 cm × 8 cm, with other lesions on the hands, soles, calves, thighs, and back. No induration of nerves could be palpated.
Figure 1:
Clinical manifestations of leprosy (Hansen’s Disease) in the patient. Figure courtesy of Samuel Bourassa-Blanchette
Other relevant investigations revealed a normal hemoglobin A1c, negative serology for hepatitis C, HIV, and syphilis, a normal immune panel except for mildly elevated erythrocyte sedimentation rate (ESR), and weak positive cryoglobulins. A percutaneous skin biopsy was performed.
Diagnosis
In clinic, there was a strong suspicion that her symptoms were related to Hansen’s Disease. Skin biopsy showed dermal granulomas with scant acid-fast bacilli, and polymerase chain reaction (PCR) testing for Mycobacterium leprae was positive. Bacillary loading was not available from skin biopsies; however, the patient was given a diagnosis of multibacillary borderline lepromatous.
Discussion
Leprosy is caused by M. leprae. It involves the skin and peripheral nerves. Incidence and prevalence are underestimated due to challenges in diagnosis (1) and unfamiliarity with the disease in Canada (2,3).
Manifestations can range from tuberculoid leprosy to lepromatous leprosy depending on the patient’s immune response (4) Skin lesions are typically erythematous and/or hypopigmented with four typical patterns: macules, plaques, infiltrative lesions, and subcutaneous nodules with or without neurologic symptoms (1). Untreated, the disease can lead to nerve damage and loss of function. Molecular testing by PCR of M. leprae DNA in skin biopsies is highly accurate and sensitive and allows for rapid detection.
Although hepatitis B cryoglobulinemia has been reported in the remote literature, further studies have failed to illustrate that it is a significant cause of cryoglobulinemia (5) We present a case of Hansen’s Disease in which there was delay in diagnosis and treatment. Retrospective analysis of the case identified that anchoring to the initial diagnosis was one of the reasons for delay (6) Leprosy should be high on the differential diagnosis in patients presenting with longstanding skin lesions and peripheral neuropathy, particularly if they have resided in an endemic area, such as South America and the Philippines. Hansen’s Disease presents as a spectrum that can range from tuberculoid leprosy to lepromatous leprosy, which can make diagnosis challenging.
Funding Statement
Publication of this article was funded by the Association of Medical Microbiology and Infectious Disease (AMMI) Canada.
Competing Interests:
The authors have nothing to disclose.
Ethics Approval:
N/A
Informed Consent:
N/A
Registry and the Registration No. of the Study/Trial:
N/A
Animal Studies:
N/A
Funding:
Publication of this article was funded by the Association of Medical Microbiology and Infectious Disease (AMMI) Canada.
Peer Review:
This article has been peer reviewed.
References
- 1.Renault CA,Ernst JD. Mycobacterium leprae (leprosy). In:Bennett JE,Dolin R,Blaser MJ, editors. Mandell, Douglas, and Bennett’s principles and practice of infectious diseases. 8th ed. Philadelphia: Elsevier Saunders; 2015. p. 2819–31. [Google Scholar]
- 2.Boggild AK,Keystone JS,Kain KC. Leprosy: a primer for Canadian physicians. CMAJ. 2004;170(1):71–8. Medline:. [PMC free article] [PubMed] [Google Scholar]
- 3.Bonnar PE,Cunningham NP,Boggild AK, et al. Leprosy in nonimmigrant Canadian man without travel outside North America, 2014. Emerg Infect Dis. 2018;24(1):165–6. 10.3201/eid2401.170547. Medline: [DOI] [PMC free article] [PubMed] [Google Scholar]
- 4.Jacobson RR,Krahenbuhl JL. Leprosy. Lancet. 1999;353(9153):655–60. 10.1016/S0140-6736(98)06322-3. Medline: [DOI] [PubMed] [Google Scholar]
- 5.Bonomo L,Dammacco F,Meneghini C,LoSpalluto M. Cryoglobulinemia in lepromatous leprosy: an immune complex phenomenon. Int J Lepr Other Mycobact Dis. 1971;39(2):554–5. Medline: [PubMed] [Google Scholar]
- 6.World Health Organization (WHO). Global leprosy: update on the 2012 situation [Internet]. Geneva: WHO; 2013;88(35):365–80. Available from: https://www.who.int/wer/2013/wer8835/en/. [PubMed] [Google Scholar]