doi: 10.1111/1346-8138.12406
Journal of Dermatology 2014; 41: 343–345
CONCISE COMMUNICATION
Case of lepromatous leprosy misdiagnosed as systemic sclerosis Jin Yong LEE, So Eun PARK, Soo Jung SHIN, Chul Woo KIM, Sang Seok KIM Department of Dermatology, Kangdong Sacred Heart Hospital, Hallym University College of Medicine, Seoul, Korea
ABSTRACT Hansen’s disease (HD) is a chronic granulomatous infectious disease caused by Mycobacterium leprae. The worldwide prevalence rate of HD has decreased gradually over the years. The clinical manifestations of HD are extensive, with involvement of the skin and various organs, and these can resemble those of many rheumatic diseases. Our patient initially presented with gradual sclerotic skin change and slight sclerodactyly with Raynaud’s phenomenon, which is frequently observed in systemic sclerosis. However, a skin biopsy with acid-fast stain later confirmed lepromatous leprosy. We report this case to emphasize the role of dermatologists for applying a systematic approach to the skin lesions of HD, which has become difficult to detect because of its rapidly declining prevalence rate.
Key words:
Hansen’s disease, lepromatous leprosy, sclerodactyly, scleroderma, systemic sclerosis.
INTRODUCTION Although Hansen’s disease (HD) was prevalent in Korea, the number of new patients drastically dropped from 499 in 1980 to 32 (31 patients from Korea and one from Bangladesh) in 2012 because of successful disease control.1 Thus, owing to its recent low prevalence rate and various clinical aspects, an early HD diagnosis is considered fairly difficult for physicians other than well-experienced dermatologists. Herein, we report a case of lepromatous leprosy that was misdiagnosed as systemic sclerosis (SSc) because of sclerotic skin and sclerodactyly with Raynaud’s phenomenon, which commonly occur in SSc.
CASE REPORT An 82-year-old Korean woman noticed several erythematous plaques on her extremities and experienced hemorrhagic necrosis accompanied by continuous hypoesthesia. The patient was thought to have Buerger’s disease and was receiving treatment for the hypoesthesia, which had begun 6 years before presentation. She presented to the hospital with the chief complaint of ulcerative changes and repetitive hypoesthesia in the limbs as well as sclerotic skin change, which had started approximately 3 years before presentation. A week before hospitalization, the patient experienced general symptoms such as fever, malaise, and edema in the lower limbs along with hemorrhagic necrosis at the digit tips. The patient had been complaining of discoloration of toes and fingers for
approximately 1 year, indicating Raynaud’s phenomenon. After hospitalization, she was tested for suspected SSc in the department of rheumatology and also underwent a biopsy by the department of dermatology. On physical examination, both palms had several erythematous plaques with shiny surfaces and slight protuberant edges, while the digit tips had hemorrhagic necrosis (Fig. 1a). Because the patient could not feel a pinprick or thermal sensation, we suspected that the asymptomatic ulcerative necrosis in her fingers was caused by neurological deficit rather than vascular occlusion. The stiff, bent fingers caused by limited joint movements indicated slight sclerodactyly, especially in distal interphalangeal joints. It seemed to be in the course of progression to claw hands. Overall, on palpation, hard, rough, dry sclerotic lesions were noted on the hands and arms to the legs and feet, and edema was present in the lower limbs (Fig. 1b). No sharply demarcated multiple nodules and papules resembling dermatofibroma or histiocytoma were present, which are commonly observed in lepromatous leprosy and erythema nodosum leprosum. A review of systems for ocular involvement, ear lobule enlargement, nasal congestion, and hair loss in any part of the body was unremarkable. Routine laboratory investigation results were all within normal range, except for erythrocyte sedimentation rate (39 mm/h; normal,
Journal of Dermatology 2014; 41: 343–345
CONCISE COMMUNICATION
Case of lepromatous leprosy misdiagnosed as systemic sclerosis Jin Yong LEE, So Eun PARK, Soo Jung SHIN, Chul Woo KIM, Sang Seok KIM Department of Dermatology, Kangdong Sacred Heart Hospital, Hallym University College of Medicine, Seoul, Korea
ABSTRACT Hansen’s disease (HD) is a chronic granulomatous infectious disease caused by Mycobacterium leprae. The worldwide prevalence rate of HD has decreased gradually over the years. The clinical manifestations of HD are extensive, with involvement of the skin and various organs, and these can resemble those of many rheumatic diseases. Our patient initially presented with gradual sclerotic skin change and slight sclerodactyly with Raynaud’s phenomenon, which is frequently observed in systemic sclerosis. However, a skin biopsy with acid-fast stain later confirmed lepromatous leprosy. We report this case to emphasize the role of dermatologists for applying a systematic approach to the skin lesions of HD, which has become difficult to detect because of its rapidly declining prevalence rate.
Key words:
Hansen’s disease, lepromatous leprosy, sclerodactyly, scleroderma, systemic sclerosis.
INTRODUCTION Although Hansen’s disease (HD) was prevalent in Korea, the number of new patients drastically dropped from 499 in 1980 to 32 (31 patients from Korea and one from Bangladesh) in 2012 because of successful disease control.1 Thus, owing to its recent low prevalence rate and various clinical aspects, an early HD diagnosis is considered fairly difficult for physicians other than well-experienced dermatologists. Herein, we report a case of lepromatous leprosy that was misdiagnosed as systemic sclerosis (SSc) because of sclerotic skin and sclerodactyly with Raynaud’s phenomenon, which commonly occur in SSc.
CASE REPORT An 82-year-old Korean woman noticed several erythematous plaques on her extremities and experienced hemorrhagic necrosis accompanied by continuous hypoesthesia. The patient was thought to have Buerger’s disease and was receiving treatment for the hypoesthesia, which had begun 6 years before presentation. She presented to the hospital with the chief complaint of ulcerative changes and repetitive hypoesthesia in the limbs as well as sclerotic skin change, which had started approximately 3 years before presentation. A week before hospitalization, the patient experienced general symptoms such as fever, malaise, and edema in the lower limbs along with hemorrhagic necrosis at the digit tips. The patient had been complaining of discoloration of toes and fingers for
approximately 1 year, indicating Raynaud’s phenomenon. After hospitalization, she was tested for suspected SSc in the department of rheumatology and also underwent a biopsy by the department of dermatology. On physical examination, both palms had several erythematous plaques with shiny surfaces and slight protuberant edges, while the digit tips had hemorrhagic necrosis (Fig. 1a). Because the patient could not feel a pinprick or thermal sensation, we suspected that the asymptomatic ulcerative necrosis in her fingers was caused by neurological deficit rather than vascular occlusion. The stiff, bent fingers caused by limited joint movements indicated slight sclerodactyly, especially in distal interphalangeal joints. It seemed to be in the course of progression to claw hands. Overall, on palpation, hard, rough, dry sclerotic lesions were noted on the hands and arms to the legs and feet, and edema was present in the lower limbs (Fig. 1b). No sharply demarcated multiple nodules and papules resembling dermatofibroma or histiocytoma were present, which are commonly observed in lepromatous leprosy and erythema nodosum leprosum. A review of systems for ocular involvement, ear lobule enlargement, nasal congestion, and hair loss in any part of the body was unremarkable. Routine laboratory investigation results were all within normal range, except for erythrocyte sedimentation rate (39 mm/h; normal,
