1/4. Vasculitic neuropathy in a patient with inactive treated lepromatous leprosy.A 46 year old Asian male with previously treated lepromatous leprosy developed a stepwise multifocal sensory disturbance 25 years later. neurophysiology demonstrated marked deterioration from previous studies. sural nerve biopsy disclosed a vasculitic process superimposed on inactive lepromatous leprosy. Immunocytochemical stains for mycobacterial antigen showed deposits within nerve and vessel walls. A delayed vasculitic neuropathy precipitated by persisting mycobacterial antigen is proposed.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/4. 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)- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/4. Clinical and histologic variations among thirty patients with Lucio's phenomenon and pure and primitive diffuse lepromatosis (Latapi's lepromatosis).The clinical and histologic experience with 30 patients who had Lucio's phenomenon, and pure and primitive diffuse lepromatosis (Latapi's lepromatosis) has been reviewed. The unanticipated clinical findings were a male to female ratio of nearly 1:1, a 21 month median time of onset of erythema nodosum leprosum (Type 2 reaction) after starting antibacterial treatment, and an absence of a stocking-glove pattern of anesthesia in 7 patients. The only unanticipated histologic finding was a lepromatous-granulomatous vasculitis, occurring in comparatively large vessels, or in vessels made large by pathologic changes, located near the dermal-subcutaneous interface. This finding was present in 6 of the 22 patients with histologic material available for review. In 2 of these 6 this vasculitis was identified before the onset of Lucio's phenomenon. With one conspicuous exception, the onset of treatment with a microbicidal agent was associated with a cessation of new lesions of Lucio's phenomenon within one week. Long-term morbidity, other than Type 2 reaction, was found in 22 of the 25 patients followed for more than 1.3 years. Usually this was the consequence of Latapi's lepromatosis, specifically venous insufficiency and/or loss of protective sensation, and only rarely from Lucio's phenomenon, specifically scar formation. Briefly summarized are the seven patients who had had a skin biopsy before the onset of Lucio's phenomenon, as well as the two patients who were considered to be atypical. Criteria for the diagnosis of Latapi's lepromatosis, in the absence of Lucio's phenomenon, are also considered.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
4/4. Polychromatic corneal and conjunctival crystals secondary to clofazimine therapy in a leper.A 67-year-old man had a diagnosis of dapsone-resistant lepromatous leprosy. He received clofazimine (Lamprene) at a dosage of 100 mg twice daily. After 3 years of therapy, results of slit-lamp examination disclosed myriad polychromatic crystals diffusely involving the cornea and perilimbal conjunctiva of both eyes. Thick sections (1 micron) from a conjunctival biopsy showed numerous rectangular-to-rhomboidal crystals within stromal fibroblasts and macrophages. By electron microscopy, these cells contained elongated, membrane-bound, cleft-like spaces that corresponded to the sites where crystals had been present previously. Additionally, complex lipid inclusions were observed in mesenchymal cells as well as in endothelial cells and pericytes of blood vessels. The ocular side effects of clofazimine therapy are reviewed. clofazimine-induced keratopathy should be included in the differential diagnosis of patients with polychromatic crystalline deposits in the corneas. To the best of the authors' knowledge, this complication of clofazimine therapy has not been described previously.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |