11/2496. Systemic sarcoidosis presenting with multiple tattoo granulomas and an extra-tattoo cutaneous granuloma. We describe a 29 year old Caucasian man who developed cutaneous sarcoidosis manifesting itself as a tumour at the left outer canthus clinically mimicking a basal cell carcinoma and nine tattoo granulomas. Subsequent investigation revealed that the patient was also suffering from systemic sarcoidosis. ( info) |
12/2496. Systemic granulomatous arteritis associated with Epstein-Barr virus infection. A 61-year-old woman initially presented with symptoms and findings reminiscent of infectious mononucleosis, and her illness then took a rapidly fatal course. autopsy revealed widespread granulomatous arteritis, with multinucleated giant cells but without eosinophils and fibrinoid necrosis, affecting small arteries and arterioles and infiltration of haemophagocytic histiocytes into many organs. in situ hybridization with Epstein-Barr virus (EBV)-specific oligonucleotide probes showed positive signals in the infiltrating immune cells and epithelial and endothelial cells of the affected organs. EBV-associated haemophagocytic syndrome (EBV-AHS) with systemic granulomatous arteritis was diagnosed. From the immunophenotypes of the infiltrating immune cells, a possible role of CD4 T-cells in the pathogenesis of this haemophagocytic syndrome and granulomatous vasculitis was suggested. ( info) |
13/2496. Double cancers of the lung and esophagus associated with a sarcoid-like reaction in their regional lymph nodes: report of a case. A case of double cancers of the lung and esophagus associated with a sarcoid-like reaction in their regional lymph nodes is reported. A 73-year-old man with hemosputum was found to have a mass in his right lower lung field on a chest X-ray. Based on a diagnosis of lung cancer, a right middle and lower lobectomy with a dissection of the lymph nodes was performed. Microscopically, a well developed granulomatous reaction was seen in the dissected mediastinal and hilar lymph nodes. Three years after the pulmonary resection, he was admitted to our hospital because of dysphagia. A diagnosis of lower esophageal cancer was made. A lower esophagectomy with a total gastrectomy was performed. A sarcoid-like reaction comprising epithelioid cells and giant cells was seen in the regional lymph nodes. No clinical findings indicative of systemic sarcoidosis were observed. This rare condition may therefore help to improve our overall understanding of the relationship between malignant neoplasms and sarcoid-like reactions in the regional lymph nodes. ( info) |
14/2496. Histopathology and electron and immunofluorescence microscopy of gingivitis granulomatosa associated with glossitis and cheilitis in a case of Anderson-fabry disease. A 17-year-old white boy with signs, symptoms, and family history of angiokeratoma corporis diffusum universale, Anderson-fabry disease (AFD), developed recurrent and then persistent swelling of both lips, erythematous hyperplastic gingivae, and a pebbled tongue. Positive blood findings were raised serum IgE, decreased T-cell level, and increased B-cell level. Histopathology of the gingiva showed noncaseating granulomas with multinucleate giant cells containing Schaumann bodies and large plasma-cell infiltrates in which immunofluorescence demonstrated immune globulins of several classes. Electron microscopy and histochemistry demonstrated ceramide in the vasculature. No glycolipid was found in the macrophages or giant cells of the granulomas which, in contrast, resembled sarcoid reactions. plasma cells with Russell bodies and immune reaction-induced degranulation of mast cells were also identified. The pathogenesis of the oral findings possibly relates to altered immune reactivity associated with damage to the microvasculature analogous to that in melkersson-rosenthal syndrome. ( info) |
15/2496. Pars plana vitrectomy and subretinal surgery for ocular toxocariasis. The clinical course of ocular toxocariasis and the chronological development of peripheral retinal and macular granulomas are reported. Removing the epiretinal as well as subretinal component of the granuloma via pars plana vitrectomy and retinotomy techniques yielded an excellent clinical result. Clinicopathologic correlation of the specimen confirmed the diagnosis with histological evidence of degenerated larval structures in granulomatous inflammation. ( info) |
16/2496. Intravenous injection of talc-containing drugs intended for oral use. A cause of pulmonary granulomatosis and pulmonary hypertension. Clinical and morphologic features are described in two patients known to have repeatedly injected intravenously talc-containing drugs intended for oral use. In one patient severe pulmonary hypertension developed; the talc granulomas in him were located predominantly within the pulmonary arteries. The second patient had normal pulmonary arterial pressures, and the talc granulomas in him were located predominantly in the pulmonary interstitium. Of 19 previously described patients with pulmonary talc granulomas, 12 had morphologic evidence of pulmonary hypertension (in three of severe degree); in each, talc granulomas were located predominantly within the pulmonary arteries. In those without signs of pulmonary hypertension, granulomas were located predominantly in the pulmonary interstitium. Why there are differences in the distribution of the talc granulomas is unclear. It is clear, however, as demonstrated by one of our patients, that severe pulmonary hypertension may be a consequence of intravenous injection of drugs intended for oral use. ( info) |
17/2496. Pulmonary small cell carcinoma associated with sarcoid reactions: report of a case. A 45-year-old male was admitted to our hospital for investigation of a nodular shadow in segment 5 of the right lung on a chest computed tomogram. A right middle lobectomy with mediastinal lymph node dissection was performed under a diagnosis of lung cancer, and histologic examination confirmed small cell carcinoma. There were sarcoid reactions in the resected lymph nodes and the lung parenchyma, but no signs of systemic sarcoidosis were evident. Sarcoid reactions are rarely observed in the regional lymph nodes draining malignant tumors. Moreover, while they are most common in squamous cell carcinoma of the lung, they extremely rare in small cell carcinoma. To our knowledge, this is only the third report of this unusual entity in the English and Japanese literature. ( info) |
18/2496. Cutaneous granulomas associated with high-grade T-cell non-Hodgkin's lymphoma. We describe a patient with non-Hodgkin's lymphoma treated with a chemotherapy regimen (which included bleomycin, prednisolone and low-dose methotrexate) who developed cutaneous non-caseating granulomas. We felt our patient fitted into the category of 'sarcoid-like granulomas'. ( info) |
19/2496. Granulomatous mycosis fungoides: report of a case with some histopathologic features of granulomatous slack skin. We describe a case of granulomatous mycosis fungoides, tumor stage, mimicking sarcoidosis in an 82-year-old man with a 2-year history of skin disease. The final diagnosis was established after one of seven biopsy specimens showed a nongranulomatous histologic picture of patch-stage mycosis fungoides. Monoclonality was proven for the lymphocytic population by T-cell-receptor rearrangement studies. The unusually extensive granulomatous inflammation with huge giant cells surrounded by CD1a-positive cells in the other six biopsy specimens was suggestive of the histopathology of granulomatous slack skin, another rare granulomatous cutaneous T-cell lymphoma. Because both a clinical and histologic overlap between granulomatous mycosis fungoides and granulomatous slack skin have been reported in the literature, we conclude that they may belong to the spectrum of a single disease. ( info) |
| Granulomatous hepatitis associated with coxiella burnetii acute infection has an adverse clinical course in some patients. Surprisingly, it does not respond to antibiotic but to steroids treatment. A hypersensitivity mechanism has been implicated. A case of granulomatous hepatitis complicating C. burnetii acute infection is reported, which was refractory to antibiotics but, as in four other cases previously reported, showed a complete response to steroids. This case was found to support findings that moderate doses of steroids can be useful in patients with granulomatous hepatitis complicating C. burnetii infection and showing no response to antibiotic treatment. ( info) |