Crystal-storing histiocytosis due to massive accumulation of charcot-leyden crystals: a unique association producing colonic polyposis in a 78-year-old woman with eosinophilic colitis.

Crystal-storing histiocytosis is a rare diagnosis that to date has only been associated with 2 conditions: intracytoplasmic accumulation of crystallized immunoglobulins in patients with lymphoproliferative disorders or plasma cell dyscrasias, and histiocytic accumulations of phagocytosed clofazimine, a drug used to treat lepromatous leprosy. We describe a 78-year-old woman with a past medical history of dermatologic mastocytosis and peripheral eosinophilia who presented with diarrhea and weight loss, and was found at colonoscopy to have polyposis limited to the right and transverse colon. She eventually underwent subtotal colectomy to remove the segment of polyposis. At gross examination, the colonic mucosa contained numerous polyps ranging from 1 to 7 mm which on histologic evaluation proved to represent mucosal and submucosal collections of histiocytes whose cytoplasm was distended by numerous brightly eosinophilic crystals. An intense eosinophilic infiltrate surrounded the histiocyte collections and also mildly involved the intervening colonic mucosa and superficial submucosa. Electron microscopy confirmed the presence of intracytoplasmic material identical to Charcot-Leyden crystals within histiocytes, representing the breakdown products of degranulated eosinophils. This is the first reported case of crystal-storing histiocytosis produced by massive accumulation of Charcot-Leyden crystals in eosinophilic colitis.

Ultrastructure of leprous phlebitis.

An ultrastructural study on vein biopsies from six lepromatous leprosy patients was carried out. The results showed that a) the lumen-lining bacillated cells were endothelial in origin due to the presence of specific Weibel-Palade endothelial cell granules; b) endothelial cells released Mycobacterium leprae into the lumen by exophagocytosis; c) M. leprae were able to grow and multiply in the endothelial and smooth muscle cells; and d) smooth muscle cells did not show any evidence of reaction due to the presence of M. leprae in their cytoplasm.

Electron microscopic observations of intracytoplasmic inclusions in human and murine leprosy bacilli.

The intracytoplasmic inclusions of Mycobacterium leprae in human lepromata and M. lepraemurium in murine lepromata were studied in ultrathin serial sections at the electron microscopic level. The inclusions were mostly homogeneous and spherical, and did not exist uniformly throughout the bacillary cells. They did not appear to be delimited by membranous structures and apparently had no internal structure. There seemed to be fundamentally little difference between M. leprae and M. lepraemurium in the fine structure of these inclusions. However, the large diffuse inclusions observed in the cells of M. lepraemurium may be a special feature of murine bacilli.

[Hansen's neuritis in the form of pseudysyringomyelia. Clinical, diagnostic and therapeutic consideration a propos of 3 cases]. / Névrite hansénienne à forme pseudosyringomyélique. Considérations cliniques, diagnostiques et thérapeutiques à propos de trois cas

Three cases of neuropathic leprosy (one Moroccan, two non-colonial immigrants) enable attention to be drawn to two aspects of theproblem of Hansen's neuritis: 1) such cases should no longer be considered exceptional, infection occuring in countries where leprosy is endemic but the disease developing years later in any country; 2) among the various neurological aspects of neuritis due to Hansen's bacillus, great stress is laid on sensory disorders of the syringomyelic type, with thermalgesic dissociation; although these symptoms have been considered typical (our three cases are an illustration of this), they seem to occur fairly rarely and are the cause of difficulties in diagnosis as it is the last thing one would think of. Treatment of these neural forms is not encouraging, in spite of a therapeutic arsenal which in theory is considered effective.