Published Web Locationhttps://doi.org/10.5070/D32h26b6ww
Lepromatous leprosyDepartment of Dermatology, New York University
Matthew J Mahlberg MD, William R Levis MD
Dermatology Online Journal 14 (10): 27
Lepromatous leprosy is a form of chronic granulomatous disease that is caused by infection with Mycobacterium leprae. Early involvement is marked by widespread, ill-defined, erythematous papules and plaques. With early intervention, leprosy is a curable disease; however, if not recognized and treated promptly, permanent sequelae and disability result. We present a patient with long-standing lepromatous leprosy who exhibits many of these sequelae.
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A 55-year-old man presented to the Hansen Disease Clinic at Bellevue Hospital Center in March, 2008, with a several-decade history of lepromatous leprosy with progressive peripheral neuropathy in addition to facial and extremity deformities. Originally from Burma, the patient recalled having erythematous patches on his face and torso in the 1990s and began to develop distal extremity and facial deformities several years later. He complained of a progressive loss of peripheral sensation, which extended to the elbows and knees. He had been treated for these findings from 2002 until 2007 in Thailand with clofazamine, dapsone, and rifampin as noted in a treatment diary. Past medical history includes tuberculosis, which the patient said had been treated, and chronic hepatitis B infection. There was no history of diabetes mellitus or human immunodeficiency virus infection. At the time of initial presentation, the patient was not taking any medications.
There were diffuse, scaly patches on the abdomen and all four extremities. Saddle-nose deformity was present and eyebrows were absent. Both hands demonstrated contraction deformities as well as the loss of distal digits. Sensation to pain and temperature was absent distal to the elbows on both arms and distal to the knees on both legs. Ulcers were present on the left first metatarsal head, right great toe, and right medial malleolus.
Hemoglobin was 10.8 g/dL (range 14 to 18) with normal iron, ferritin, and total iron binding capacity levels. Erythrocyte sedimentation rate was 54 mm/hr (range 0 to 15), follicle stimulating hormone 35.9 U/L (range 1.0 to 14.0), luteinizing hormone 25.02 U/L (range 1.5 to 9.3), total testosterone 237 ng/dL (range 260 to 1000), and free testosterone 21.7 pg/mL (range 50 to 210). Glucose-6-phosphate dehydrogenase was normal. Sputum acid-fast bacilli stain was negative and a chest radiograph was unremarkable.
Leprosy, also known as Hansen disease, is a chronic, granulomatous disease caused by the obligate intracellular organism Mycobacterium leprae. Though seldom lethal, the disease causes appreciable morbidity principally through its effects on the skin, peripheral nerves, and nasal mucosa . Due to international travel, leprosy can be found anywhere in the world; in 2005, 166 new cases were reported in the United States . However, the disease remains primarily in endemic regions of the world, which include Brazil, India, Madagascar, Mozambique, Myanmar, and Nepal, the six countries with the highest incidence .
To characterize the clinical spectrum of disease, two classification systems for leprosy have been devised. The WHO classifies patients as paucibacillary (PB) if there are five or fewer skin lesions or multibacillary (MB) if there are six or more skin lesions or if a skin smear is positive. The Ridley-Jopling classification correlates the number of bacilli found in the dermis with clinical findings and immunologic mechanism . These classification systems are utilized in determining treatment regimens and predicting clinical outcome in patients.
The clinical characteristics of leprosy vary by subtype and primarily affect the skin and nervous system. Lepromatous leprosy, as seen in this patient, has the greatest number of bacilli and is characterized by early, widespread, ill-defined, erythematous or hypopigmented papules and plaques. Later findings include facial infiltration (leonine facies), ocular effects, neurologic involvement, and hypogonadism .
Severe peripheral neuropathy in our patient resulted in peripheral anesthesia, distal digit resorption, and claw-hand deformity. Neurologic involvement may be detected early by nerve tenderness or enlargement. Neurologic damage appears to be multifactorial and is caused by three general mechanisms: Schwann cell damage due to M. leprae; damage due to inflammatory and immune-mediated processes; and damage due to edema and mechanical processes, such as compression [6, 7]. Patients should be followed closely for development or progression of neuropathy, with serial skin and neurologic examinations before, during, and after treatment for leprosy .
Other notable clinical findings in this patient with lepromatous leprosy include saddle- nose deformity and hypogonadism. Saddle-nose deformity results from infiltration of the nasal mucosa and bone . Hypogonadism is indicated by low levels of total and free testosterone as well as elevated follicle stimulating hormone (FSH) and luteinizing hormone levels, which have been associated with lepromatous leprosy. These hormonal abnormalities are not associated with borderline-tuberculoid leprosy. FSH levels appear to correlate with disease duration .
To avoid sequelae, early recognition and treatment are necessary. Multi-drug therapy regimens have been devised and recommended by the WHO and are based on PB or MB status. These medications include rifampin, clofazimine, and dapsone and are given for six months to two years, depending on the clinical findings . After appropriate therapy, patients are no longer infectious to others and typically do not develop the chronic symptoms of the disease, especially when diagnosed and treated at the earliest stages. However, it is important to follow patients even after therapy has ended to monitor for late sequelae because these are difficult to treat and often are permanent. Extension of treatment beyond the standard WHO guidelines may be necessary when persistent or late sequelae are identified.
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