DEPARTMENT OF PATHOLOGY
The Johns Hopkins Medical Institutions



Vol. 20, No. 10
THE JOHNS HOPKINS MICROBIOLOGY NEWSLETTER
Tuesday, March 6, 2001
 

  1. Provided by Leslie Edwards Reger, Division of Outbreak Investigation, Maryland Department of Health and Mental Hygiene.

  2.  

     

    No information reported
     

  3. The Johns Hopkins Hospital. Information provided by Bahram Robert Oliai M.D., Department of Pathology.
Case Report: The patient is a 29 year old Indian woman with an approximately six month history of worsening numbness and pain. She reported that her symptoms started with mild parathesias of the left hand and blister like sores. These symptoms resolved for a short period and recurred with tingling and numbness of the left index finger. At this point she sought treatment (in India). Physical examination was additionally significant for several areas of discoloration on the right foot. A biopsy was performed to rule out leprosy which was read as negative, however given her clinical presentation she was started and maintained on anti-lepromatous therapy (Dapsone 100 mg q.d., Hanispran q.o.d., and Rifampin q 15 days). During her course of treatment she suffered several adverse reactions including hyperpigmentation, malaise, poor appetite, weight loss, and rash. In addition she developed increasing numbness in the right and left hands. Subsequently she referred herself to The Johns Hopkins Hospital for evaluation and further treatment. Physical examination was most significant for two well demarcated areas of hypopigmentation on the right foot and a hyperpigmented rash, most prominent on the right ankle. Neurologic examination demonstrated a profound sensory loss in the left hand with a relative sparing of the thumb and a decrease in sensation to pinprick in the ulnar distribution of the right hand. Palpable, ropey, peripheral nerves were noted, especially prominent at the left elbow. Her clinical presentation was thought to be consistent with tuberculoid leprosy and a biopsy was performed of the right sural nerve. Microscopic examination demonstrated marked granulomatous inflammation. Stains for acid fast bacilli were negative.
Mycobacterium leprae/Hansen’s Disease
Organism: The causative mycobacterium of leprosy is Mycobacterium leprae. It is an obligate intracellular organism, multiplies very slowly, and has yet to be cultivated successfully in vitro. M. leprae grows best at 27-30 degrees C, hence its predilection for peripheral nerves and skin in the cooler parts of the body. Worldwide registered cases have fallen from 5.4 million (1985) to approximately 1 million. The majority of cases are in developing countries with the highest numbers in India and Brazil. In the United States most cases involve patients who have lived in foreign countries, however Hansen’s Disease is endemic in Texas, Hawaii, Louisiana, and California. M. leprae is found in wild armadillos in the south-central United States. Transmission is most probably via the respiratory route since the nasal discharge from patients with untreated multibacillary Leprosy often contains large numbers of bacilli. Cases due to exposure to infected armadillos have occurred.

Clinical Manifestations: Standard nomenclature divides cases into five groups based on clinical, histopathologic, and immunologic findings: tuberculoid, borderline tuberculoid, borderline, borderline lepromatous, and lepromatous. The tuberculoid form of disease is most common in those with a vigorous immune response. In these patients infection is limited to a few places in the skin and peripheral nerves, manifesting with 1-6 erythematous plaques with sharp borders on the face, trunk, or extremities. These plaques are hairless, dry, and hypoesthetic. The number of bacteria in these lesions is low and they are therefore referred to as paucibacillary. Nerves may be palpably thickened secondary to inflammation in tuberculoid Hansen’s Disease (see Figure 1). Common nerves affected include: the great auricular nerve, common peroneal nerve, ulnar nerve, radial cutaneous nerve and the median nerve. At the other end of the spectrum, patients with minimal cellular immune response have lepromatous disease. Skin involvement is extensive with numerous bilateral macules and papules. The bacteria can infect the nasal mucosa resulting in stuffiness, discharge, epistaxis, and destruction of the nasal cartilage. Diffuse infiltration of the skin with bacteria results in the classic leonine facies (see Figure 2). Unusual presentations include erytema nodosum like leprosum (similar to erythema nodosum) and a form of lepromatous disease reported primarily in Mexican patients with diffuse infiltration and thickening of the skin, loss of eyebrows and body hair, and a necrotizing vasculitis termed "Lucio’s phenomenon."


Figures 1 (left) and 2 (right). Figure 1 shows nerve thickening in tuberculoid leprosy (arrow, great auricular nerve). Figure 2 shows the "leonine facies" of lepromatous leprosy. From Forbes CD, Jackson WF. A Color Atlas and Text of Clinical Medicine. Wolfe Publishing, Aylesbury, England, 1st ed, 1993, pp. 49-50.

Diagnosis: A skin lesion that is hypoesthetic, hairless, or dry should raise the suspicion for Hansen’s disease. The diagnosis is generally made by the findings of the classic histopathologic changes or acid fast bacilli in skin or nerve biopsies. Lepromatous and borderline lesions will usually have many organisms and tuberculoid few, if any. In such situations the diagnosis must be made on clinical ground, although the finding of a granulomatous dermatitis/neuritis supports the diagnosis.

Treatment: Treatment regimens are summarized in Table 1. In addition to multidrug regimens studies have suggested that BCG inoculation protects against leprosy. A single dose appears to be 50% protective and two doses may increase the protection further.


Table 1. Recommended Treatment Regimens for Hansen’s Disease. From Wathen PI. Hansen’s Disease. Southern Medical Journal 1996;98(7):647-652.

References:

1. Forbes CD, Jackson WF. A Color Atlas and Text of Clinical Medicine. Wolfe Publishing, Aylesbury, England, 1st ed, 1993, pp. 49-50.

2. Jacobsen RR, Krahenbuhl JL. Leprosy. The Lancet 1999;353:655-660.

3. Wathen PI. Hansen’s Disease. Southern Medical Journal 1996;98(7):647-652.

4. Whittey CJM, Lockwood DNJ. Leprosy – New Perspectives on an Old Disease. Journal of Infection 1999;38:2-5.


Help | Feedback | Pathology Home | Previous Menu | Tool Box* | Search
Copyright © 2001 THE JOHNS HOPKINS UNIVERSITY