Cases reported "Leprosy, Lepromatous"

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1/12. cytodiagnosis of erythema nodosum leprosum. A case report.

    BACKGROUND: The application of cytology in leprosy has been restricted to the evaluation of morphologic and bacterial indices by slit skin smears to facilitate diagnosis of cases according to the Ridley-Jopling scale. Isolated reports have now documented the use of fine needle aspiration cytology (FNAC) in the diagnosis of leprosy. CASE: A 45-year-old male presented with the abrupt onset of multiple nodular eruptions all over the body. The clinical diagnosis was Sweet's syndrome. FNAC showed numerous neutrophils in a background of foamy macrophages. Special stains revealed the presence of a large number of fragmented acid-fast bacilli in the smears. A diagnosis of erythema nodosum leprosum (ENL) was made on FNAC. CONCLUSION: The presence of neutrophils in a characteristic milieu of foamy macrophages is seen in lesions of ENL. Such a picture should prompt the cytologist to use a modified Ziehl-Neelsen stain to demonstrate acid-fast bacilli, as ENL can present as an acute episode in patients without a previous diagnosis of leprosy.
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2/12. Asymptomatic nerve hypertrophy in lepromatous leprosy: a clinical, electrophysiological and morphological study.

    In order to learn more about early nerve lesions observed in leprosy, we performed a clinical, electrophysiological and morphological study in seven patients with untreated lepromatous leprosy, palpably enlarged radial cutaneous nerve and preserved sensation in the corresponding territory. The conduction velocity of the cutaneous radial nerve, which was decreased in all patients, did not significantly differ from that of a group of patients with lepromatous leprosy, hypertrophy of the radial cutaneous nerve and sensory loss. In contrast, the sensory action potential was significantly lower in patients with sensory loss, which demonstrates that axon loss is more important than demyelination in producing sensory loss. In all patients nerve enlargement was due to thickening of the epineurium and of the perineurium subsequent to inflammatory infiltrates and proliferation of fibroblasts and perineurial cells. In several fascicles, the inflammatory infiltrates and the infected cells infiltrated endoneurial connective tissue septa and blood vessels. Mycobacteria leprae were abundant in perineurial cells, fibroblasts, macrophages, schwann cells and endothelial cells, and lymphocytic vasculitis present in all cases. The average density of myelinated fibres was 2600 SD 880 fibres/mm2 (control: 7700 fibres/mm2), with marked differences between individual fascicles, versus 420 fibres/mm2 in patients with nerve hypertrophy and sensory loss (range 0-2080 fibres/mm2). Single fibre preparations showed that segmental demyelination predominated in two patients, axonal degeneration in one, while inflammatory infiltrates and proliferation of connective tissue adhering to individual fibres were prominent in the others.(ABSTRACT TRUNCATED AT 250 WORDS)
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3/12. Leprous osteitis presenting as bone cyst and erosions.

    A 30-year-old man presented to the Hansen outpatient department with swelling and ulceration of toes for 2 months and swelling of the right fifth and fourth fingers and the left second finger for 1 month. In addition to skin lesions of lepromatous leprosy (subpolar type), there was nontender, non-fluctuant swelling of the right fifth and fourth fingers and left second finger. Skin over the right fifth finger showed sinus-like openings with associated purulent discharge. He also had swelling and ulceration of second left toe. Slit-skin smear (SSS) showed a bacterial index of 6 from the ear lobes and cutaneous nodules, 4 from the patch, and 3 from normal skin. Modified Ziehl-Neelsen staining of the discharge extruding from the sinuses on the right fifth finger also showed abundant acid-fast bacilli. radiography of the hands and feet showed lytic lesions in the distal epimetaphyseal region o proximal phalanx of the right fifth finger and left second finger and erosion of distal end of proximal phalanges of both second toes. Histopathological examination of biopsy specimen from the patch (back) showed features of lepromatous leprosy, and Fite-Faraco stain for tissue acid-fast bacteria (AFB) was strongly positive. Fine-needle-aspiration cytology (FNAC) from the lytic lesion in the bone also showed predominantly foamy macrophages with strongly positive staining for AFB with a few interspersed lymphocytes, epithelioid cells and Langhans giant cells. On the basis of these features, a clinical diagnosis of subpolar lepromatous leprosy with leprous osteitis was made. In today's clinical era of improved case detection and prompt treatment with effective multidrug regimens, advanced bone changes are rarely encountered. We describe this case of lepromatous leprosy that developed cavitating lesions of the phalanges of the hand, seen on x-ray as well-defined bone cyst and erosions.
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4/12. light and electron microscopic appearances of peripheral nerves from two lepromatous leprosy patients after 12 months of multidrug therapy and their significance.

    Biopsies from radial cutaneous nerves of a lepromatous patient and one borderline lepromatous patient treated with 12 doses of multidrug regimen were studied using light and electronmicroscopes. Histopathologically both showed typical lepromatous neuritis. Electronmicroscopic examination showed demyelination, atrophy and degeneration of myelinated axons and nonmyelinated axons and a marked increase in collagen fibrils. Perineurial cells, schwann cells and endoneurial macrophages contained numerous persisting M. leprae. Almost all the organisms in macrophages were fragmented and could be considered non-viable. A few M. leprae found in schwann cells showed structure of viable bacilli. It is possible a few dead or dormant organisms may persist for many years in schwann cells or in fibrous tissue without producing any ill effects, and may cause relapse only in rare instances. Since 12 months of MDT resulted in the clearance of M. leprae in course of time and the reported relapse rates after years were insignificant, implementation of MDT for a year for all MB patients is justified provided surveillance of these patients is ensured. Administration of uniform MDT for 6 months is worth a trial.
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5/12. Histoid leprosy - unusual presentation.

    A 39-year-old man presented with a chronic nonhealing toe ulcer and multiple skin-colored papules on the back, arms, and knees. From an initial small erosion, the toe lesion ulcerated over a 1.5-year period, while the papules progressed over a 6-month period, first appearing on the back and then spreading to the arms and knees. The past medical and family history were non-contributory. Pertinent findings included the aforementioned well-defined, asymmetric, shiny papules (Fig. 1). There were multiple, discrete, erythematous, pedunculated, tumor-like masses of various sizes over the right thigh (Fig. 2) and a well-defined 4 cm x 3 cm ulcer on the second toe of the left foot, the floor of which was covered by necrotic slough. There was distal loss of sensation to temperature, touch, and pain. The greater auricular, ulnar, radial cutaneous and common peroneal nerves were thickened bilaterally, but non-tender. A clinical diagnosis of histoid leprosy was made. The differential diagnosis for the tumor-like thigh masses included dermatofibroma, neurofibroma, and Kaposi's sarcoma. The hemogram, liver/renal function tests, chest X-ray, and abdominal ultrasound were normal. Human immunodeficiency virus enzyme-linked immunosorbent assay (hiv ELISA) was negative. The ear lobe smear (ELS) for acid-fast bacilli showed a bacterial index (BI) of 6 [> 1000 organisms/oil immersion field (oif)] and a morphological index (MI) of 50%. The skin-colored papules on the back and the pedunculated masses showed a BI of 5 (100-1,000 organisms/oif) and an MI of 50%. The normal skin showed a BI of 4 (10-100 organisms/oif) and an MI of 5%. biopsy of the papules and pedunculated tumors showed a well-circumscribed area of the dermis packed with many acid-fast organisms and foamy macrophages, consistent with histoid leprosy (Fig. 3). Fite-Faraco stain demonstrated cells packed with lepra bacilli. A final diagnosis of lepromatous leprosy, histoid variant, was made. dapsone, clofazimine, rifampicin, and, later, ofloxacin were started.
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6/12. clofazimine induced enteropathy--a case highlighting the importance of drug induced disease in differential diagnosis.

    A patient on treatment for multibacillary leprosy for the past three years, presented with episodes of abdominal pain. Since the patient improved with conservative management, clofazimine induced enteropathy was considered as a remote possibility. A review of the mucosal biopsies showed macrophages with crystal-storing spaces consistent with clofazimine deposition in the duodenum. This case highlights the need to consider and investigate drug-induced disease as part of the differential diagnosis.
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7/12. Immunophenotypic analysis of histiocytes involved in AIDS-associated mycobacterium scrofulaceum infection: similarities with lepromatous lepra.

    The present study reports a rare case of systemic M. scrofulaceum infection in an AIDS patient and analyses the inflammatory infiltrate in a lymph node by immunohistochemistry. Special emphasis is put on the histiocytes. The diffuse infiltrate consists mainly of large histiocytes that contain numerous bacilli. These cells display the phenotype of mature histiocytes and in addition coexpress the antigens recognized by RFD7 and RFD9, both markers of different subsets of histiocytes which have been reported to be co-expressed by the infected histiocytes in the infiltrate of lepromatous lepra. Interdigitating reticulum cells are rare as well as T cells which are mainly of the suppressor/cytotoxic type. These findings are similar to those reported for lepromatous lepra and might indicate common deficiencies in T cell-macrophage interactions in both conditions. Superimposed on the diffuse infiltrate of large histiocytes we observed 'monocytic granulomas', the presence of which might be related to a reactional state comparable to erythema nodosum leprosum, a reactional state of lepromatous lepra.
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8/12. skin pigmentation from clofazimine therapy in leprosy patients: a reappraisal.

    Skin biopsy specimens from two lepromatous leprosy patients with dark brown pigmentation who were receiving long-term clofazimine therapy were studied. ceroid-lipofuscin pigment was demonstrated inside macrophages that contained numerous phagolysosomes. These contained lipids and clofazimine that appeared as electron-lucent vacuoles and a lipofuscin pigment that was electron dense, granular, and lamellated. Although the presence of the drug in tissues contributed to the skin pigmentation, the main cause was a drug-induced, reversible ceroid lipofuscinosis.
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9/12. leukopenia secondary to mycobacterium leprae.

    Hansen's disease (HD) is one of the major infectious diseases in the world with an estimated total of 12 million cases. physicians in north america, however, rarely see HD or its manifestations. Hematological manifestations of HD have been reported but are not well appreciated. We report a patient with leukopenia while under treatment for active HD who demonstrated mycobacterial involvement of the bone marrow.
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10/12. Nongranulomatous involvement of the bone marrow in lepromatous leprosy.

    Bone marrow involvement in lepromatous leprosy has been characterized histologically by a proliferation of foamy histiocytes containing lepra bacilli, the so-called Virchow cells. The authors have studied three patients with biopsy-proven lepromatous leprosy in whom Fite stain, performed on histologic sections of bone marrow aspirates, demonstrated numerous bacilli lying free in the interstitium in the absence of Virchow cells or focal collections of foamy macrophages. Two of the patients had a recent diagnosis of lepromatous leprosy by skin biopsy; the third patient had a 33-year history of lepromatous leprosy that had been treated. Bone marrow aspirates were performed in all three patients for evaluation of anemia. The findings indicate that the bone marrow may act as a reservoir for viable organisms in the absence of a host response in treated and untreated patients with lepromatous leprosy. The persistence of viable organisms in the bone marrow in patients with lepromatous leprosy may account for the high rate of relapse and/or recrudescence of the disease following cessation of specific therapy. bone marrow examination with the Fite modification of the acid-fast stain is therefore indicated in such patients to evaluate bone marrow involvement and the efficacy of treatment.
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