Mid-borderline leprosy masquerading as an overlap syndrome

Mid-borderline leprosy masquerading as an overlap syndrome Rheumatology key message Patients with unusual cutaneous lesions with articular symptoms should be evaluated for leprosy. Sir, Leprosy is a chronic, infectious disease that affects multiple organs and thus presents with a myriad of clinical as well as serological manifestations. Skin, nerves and joint involvement in leprosy with false-positive serology, often simulates the rheumatic and CTDs, and the use of systemic immunosuppressive agents in view of these autoimmune diseases leads to progression to the more severe lepromatous form of leprosy. Hence, patients with unusual cutaneous and rheumatic manifestations, especially those hailing from leprosy endemic regions, should be evaluated for leprosy (Pawar M and Zawar V, unpublished work). A 59-year-old Indian male from Nashik city, Maharashtra State; a retired bank employee, presented with persistent annular erythematous plaques without scales predominantly over trunk, extremities including palms, and face for 6 months (Fig. 1). The patient complained of a burning sensation over the skin lesions after sun exposure. Four months ago, he developed joint pain (without morning stiffness) of hands (with swelling), elbows and knees, with intermittent mild fever for which he used to take ibuprofen. The patient did not have a sore throat, oral ulcers, shortness of breath or history of RP. His past and family history was unremarkable. On this occasion, he consulted a physician and his laboratory tests revealed raised ESR (40 mm/h), positive RF (25 IU/l) and ANA (1: 80, speckled pattern); otherwise, complete blood cell count, liver function tests, renal function tests, anti-CCP, anti-dsDNA, anti-U1RNP, CRP and VDRL were within normal limits. X-rays of the involved joints did not show any abnormalities. Based on the presumed diagnosis of overlap syndrome, he was prescribed oral HCQ 200 mg and prednisolone 20 mg once daily each, without much relief; hence, he was referred to us. Fig. 1 View largeDownload slide Multiple well-defined annular plaques with raised erythematous margin and clear centre almost all over body Fig. 1 View largeDownload slide Multiple well-defined annular plaques with raised erythematous margin and clear centre almost all over body Clinical examination revealed multiple erythematous annular plaques with raised borders, with evidence of slight hypoesthesia. Palms were swollen, with erythematous plaques in places. There was tenderness and swelling of wrists, MCP joints and PIP joints. Bilateral ulnar, radial and left lateral popliteal nerves were thickened. A biopsy from a lesion over the patient’s back showed diffuse epithelioid cell granulomas in the dermis and dermo-epidermal junction and around the nerves, suggestive of mid-borderline leprosy (BB). Slit skin smears (SSS) taken from the ear lobes and skin lesions were positive for acid-fast bacilli on Zeihl-Neelsen stain. The patient was put on multidrug therapy consisting of rifampicin, clofazimine and dapsone. There was significant amelioration of articular symptoms, but the skin lesions were slow to resolve. Leprosy has two main poles, a tuberculoid and a lepromatous pole. Towards the tuberculoid pole (Paucibacillary), the cell-mediated immunity of the body is intact; hence, the numbers of skin lesions are few. In contrast, towards the lepromatous pole (Multibacillary, MB), the body’s cell-mediated immunity decreases; thus, the numbers of skin lesions are numerous. Between these two poles, a broad variety of borderline forms exist, depending on the individual’s immune responses to Mycobacterium leprae. BB leprosy is an uncommon form of multibacillary leprosy. The lesions of BB vary from 10 to 20 in numbers and are present as erythematous plaques with sloping outer margins and punched-out centres, with well-defined edges, giving a Swiss cheese appearance, sometimes with streaming irregular borders, presenting a geographic appearance [1]. scLE—a subset of LE—in one-third of cases manifests as annular, polycyclic skin lesions, mainly over the trunk and simulating BB leprosy [2]. PIP, MCP, elbows and knees are the commonly involved joints in scLE, which are also the joints of predilection in leprosy patients [2, 3]. Peripheral neuropathy can be observed in certain scLE patients [2]. ANA have been reported in leprosy patients especially in the MB pole, varying from 3 to 34%, but in low titres with speckled and homogeneous pattern [4]. Interestingly, photosensitivity has also been observed in leprosy patients (Pawar M and Zawar V, unpublished work). Hence, due to cutaneous, neurologic and serological similarities, leprosy often simulates SLE. The rheumatic manifestations in the form of articular involvement are seen in 64–77% of patients with leprosy (especially those with lepra reactions). One to 5% of leprosy patients develop arthritis of the small joints of the hands, simulating RA. In 2.6–21% of leprosy patients, RF is positive; hence, leprosy patients who present with articular symptoms and RF positivity are often misdiagnosed as having RA (Pawar M and Zawar V, unpublished work) [3, 5]. It is prudent to do a thorough clinical examination, especially of the peripheral nerves, and to check the sensation of the skin lesions in patients presenting with atypical rheumatologic features and cutaneous lesions, whereas in doubtful cases, SSS and skin biopsy are often helpful. When the presenting symptoms of leprosy are those of rheumatic and CTDs, a rheumatologist is often the first to encounter such patients. As such patients are mostly of the MB pole, there is a high risk of transmission of the bacilli in society and to a treating rheumatologist as well. Hence, prompt diagnosis and treatment of leprosy is necessary in order to halt its transmission and to limit the nerve damage, thus preventing its dreaded sequelae. Funding: No specific funding was received from any bodies in the public, commercial or not-for-profit sectors to carry out the work described in this manuscript. Disclosure statement: The authors have declared no conflict of interest. References 1 Talhari C , Talhari S , Penna GO. Clinical aspects of leprosy . Clin Dermatol 2015 ; 33 : 26 – 37 . Google Scholar CrossRef Search ADS PubMed 2 Psarelis S , Georgiades F , Ioannou A et al. Subacute cutaneous lupus erythematosus inducing unilateral sensorimotor neuropathy . Rheumatol Int 2017 ; 37 : 831 – 5 . Google Scholar CrossRef Search ADS PubMed 3 Chauhan S , Wakhlu A , Agarwal V. Arthritis in leprosy . Rheumatology 2010 ; 49 : 2237 – e42 . Google Scholar CrossRef Search ADS PubMed 4 Zawar V , Kumavat S , Pawar M , Desai D. Tuberculoid leprosy masquerading as systemic lupus erythematosus: an interesting observation . Acta Dermatovenerol Alp Pannonica Adriat 2017 ; 26 : 81 – 3 . Google Scholar PubMed 5 El-Gendy H , El-Gohary RM , Shohdy KS , Ragab G. Leprosy masquerading as systemic rheumatic diseases . J Clin Rheumatol 2016 ; 22 : 264 – 71 . Google Scholar CrossRef Search ADS PubMed © The Author(s) 2018. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/about_us/legal/notices) http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Rheumatology Oxford University Press

Mid-borderline leprosy masquerading as an overlap syndrome

Rheumatology , Volume Advance Article – Apr 27, 2018

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Oxford University Press
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© The Author(s) 2018. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com
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1462-0324
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10.1093/rheumatology/key125
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Abstract

Rheumatology key message Patients with unusual cutaneous lesions with articular symptoms should be evaluated for leprosy. Sir, Leprosy is a chronic, infectious disease that affects multiple organs and thus presents with a myriad of clinical as well as serological manifestations. Skin, nerves and joint involvement in leprosy with false-positive serology, often simulates the rheumatic and CTDs, and the use of systemic immunosuppressive agents in view of these autoimmune diseases leads to progression to the more severe lepromatous form of leprosy. Hence, patients with unusual cutaneous and rheumatic manifestations, especially those hailing from leprosy endemic regions, should be evaluated for leprosy (Pawar M and Zawar V, unpublished work). A 59-year-old Indian male from Nashik city, Maharashtra State; a retired bank employee, presented with persistent annular erythematous plaques without scales predominantly over trunk, extremities including palms, and face for 6 months (Fig. 1). The patient complained of a burning sensation over the skin lesions after sun exposure. Four months ago, he developed joint pain (without morning stiffness) of hands (with swelling), elbows and knees, with intermittent mild fever for which he used to take ibuprofen. The patient did not have a sore throat, oral ulcers, shortness of breath or history of RP. His past and family history was unremarkable. On this occasion, he consulted a physician and his laboratory tests revealed raised ESR (40 mm/h), positive RF (25 IU/l) and ANA (1: 80, speckled pattern); otherwise, complete blood cell count, liver function tests, renal function tests, anti-CCP, anti-dsDNA, anti-U1RNP, CRP and VDRL were within normal limits. X-rays of the involved joints did not show any abnormalities. Based on the presumed diagnosis of overlap syndrome, he was prescribed oral HCQ 200 mg and prednisolone 20 mg once daily each, without much relief; hence, he was referred to us. Fig. 1 View largeDownload slide Multiple well-defined annular plaques with raised erythematous margin and clear centre almost all over body Fig. 1 View largeDownload slide Multiple well-defined annular plaques with raised erythematous margin and clear centre almost all over body Clinical examination revealed multiple erythematous annular plaques with raised borders, with evidence of slight hypoesthesia. Palms were swollen, with erythematous plaques in places. There was tenderness and swelling of wrists, MCP joints and PIP joints. Bilateral ulnar, radial and left lateral popliteal nerves were thickened. A biopsy from a lesion over the patient’s back showed diffuse epithelioid cell granulomas in the dermis and dermo-epidermal junction and around the nerves, suggestive of mid-borderline leprosy (BB). Slit skin smears (SSS) taken from the ear lobes and skin lesions were positive for acid-fast bacilli on Zeihl-Neelsen stain. The patient was put on multidrug therapy consisting of rifampicin, clofazimine and dapsone. There was significant amelioration of articular symptoms, but the skin lesions were slow to resolve. Leprosy has two main poles, a tuberculoid and a lepromatous pole. Towards the tuberculoid pole (Paucibacillary), the cell-mediated immunity of the body is intact; hence, the numbers of skin lesions are few. In contrast, towards the lepromatous pole (Multibacillary, MB), the body’s cell-mediated immunity decreases; thus, the numbers of skin lesions are numerous. Between these two poles, a broad variety of borderline forms exist, depending on the individual’s immune responses to Mycobacterium leprae. BB leprosy is an uncommon form of multibacillary leprosy. The lesions of BB vary from 10 to 20 in numbers and are present as erythematous plaques with sloping outer margins and punched-out centres, with well-defined edges, giving a Swiss cheese appearance, sometimes with streaming irregular borders, presenting a geographic appearance [1]. scLE—a subset of LE—in one-third of cases manifests as annular, polycyclic skin lesions, mainly over the trunk and simulating BB leprosy [2]. PIP, MCP, elbows and knees are the commonly involved joints in scLE, which are also the joints of predilection in leprosy patients [2, 3]. Peripheral neuropathy can be observed in certain scLE patients [2]. ANA have been reported in leprosy patients especially in the MB pole, varying from 3 to 34%, but in low titres with speckled and homogeneous pattern [4]. Interestingly, photosensitivity has also been observed in leprosy patients (Pawar M and Zawar V, unpublished work). Hence, due to cutaneous, neurologic and serological similarities, leprosy often simulates SLE. The rheumatic manifestations in the form of articular involvement are seen in 64–77% of patients with leprosy (especially those with lepra reactions). One to 5% of leprosy patients develop arthritis of the small joints of the hands, simulating RA. In 2.6–21% of leprosy patients, RF is positive; hence, leprosy patients who present with articular symptoms and RF positivity are often misdiagnosed as having RA (Pawar M and Zawar V, unpublished work) [3, 5]. It is prudent to do a thorough clinical examination, especially of the peripheral nerves, and to check the sensation of the skin lesions in patients presenting with atypical rheumatologic features and cutaneous lesions, whereas in doubtful cases, SSS and skin biopsy are often helpful. When the presenting symptoms of leprosy are those of rheumatic and CTDs, a rheumatologist is often the first to encounter such patients. As such patients are mostly of the MB pole, there is a high risk of transmission of the bacilli in society and to a treating rheumatologist as well. Hence, prompt diagnosis and treatment of leprosy is necessary in order to halt its transmission and to limit the nerve damage, thus preventing its dreaded sequelae. Funding: No specific funding was received from any bodies in the public, commercial or not-for-profit sectors to carry out the work described in this manuscript. Disclosure statement: The authors have declared no conflict of interest. References 1 Talhari C , Talhari S , Penna GO. Clinical aspects of leprosy . Clin Dermatol 2015 ; 33 : 26 – 37 . Google Scholar CrossRef Search ADS PubMed 2 Psarelis S , Georgiades F , Ioannou A et al. Subacute cutaneous lupus erythematosus inducing unilateral sensorimotor neuropathy . Rheumatol Int 2017 ; 37 : 831 – 5 . Google Scholar CrossRef Search ADS PubMed 3 Chauhan S , Wakhlu A , Agarwal V. Arthritis in leprosy . Rheumatology 2010 ; 49 : 2237 – e42 . Google Scholar CrossRef Search ADS PubMed 4 Zawar V , Kumavat S , Pawar M , Desai D. Tuberculoid leprosy masquerading as systemic lupus erythematosus: an interesting observation . Acta Dermatovenerol Alp Pannonica Adriat 2017 ; 26 : 81 – 3 . Google Scholar PubMed 5 El-Gendy H , El-Gohary RM , Shohdy KS , Ragab G. Leprosy masquerading as systemic rheumatic diseases . J Clin Rheumatol 2016 ; 22 : 264 – 71 . Google Scholar CrossRef Search ADS PubMed © The Author(s) 2018. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/about_us/legal/notices)

Journal

RheumatologyOxford University Press

Published: Apr 27, 2018

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