Lethal mid-line granuloma (LMG) is a clinical term, and it is histologically composed of Wegener's granulomatosis (WG), polymorphic reticulosis (PR), and malignant lymphoma (ML). WG is an inflammatory disease, and PR and ML are considered to represent a neoplastic proliferation of lymphoreticular cells. In the present report, a nation-wide study on LMG in Japan is compared with a study from the UK, to examine differences in frequencies of each disease in Eastern and Western countries. A total of 340 cases of LMG were examined. On the basis of histological and clinical findings, these cases were categorized as 68 WG, 129 PR, 92 ML, 44 chronic inflammation not specified, and 7 other related conditions. The crude frequencies of WG, PR, and ML per 100,000 outpatients of ENT clinics in Japan and England were 4, 8 and 6, respectively and 8, 4 and I, respectively. The predominance of malignant lymphoproliferative diseases to WG in Japan (3.5:1) was in marked contrast to the situation in the UK (1:1.6). A review of the pertinent literature suggests a clustering of malignant lymphoproliferative diseases of the nose among Mongolian ethnic groups.
Clinical and immunopathological analysis was performed on 24 cases of Sjögren syndrome with annular erythema (AESjS). AESjS predominantly appears on the cheek of the face where skin temperature is relatively low in comparison with other sites. VCAM-1 and ICAM-1 were strongly expressed on endothelial cells of AESjS, while epidermal expression of ICAM-1 was focal and weak. VCAM-1 mRNA expression was also much more intense compared to systemic lupus erythematosus. The lymphocyte response to staphylococcal enterotoxin B was higher in AESjS than that of controls, and cells positive for T cell receptor Vβ6,9,12 were expanded after the culture. Superantigen-driven endothelial-cell-dependent T cell infiltration to the skin plays a crucial role in AESjS.
We describe a patient with incontinentia pigmenti who developed the clinical picture of intestinal Behçet’s disease. Diagnosis of Behçet’s disease was base on a recurrent genital and oral ulcer and a positive HLA-B51. Impaired neutrophil chemotactic activity and an elevated plasma IL-6 level were found. Chromosomal study revealed no abnormality in the patient or her family. We discuss the possibility that there are common immunological abnormalities in the two syndromes.
Summary
We report lour children with anti‐Ro/SSA positive who presented with an annular erythema as a manifestation of Sjögren's syndrome. One patient had an aseptic meningoencephalitis as well as cutaneous lesions. Children with annular erythema should he carefully followed for features of Sjögren's syndrome, which is believed to be a rare condition in children.
The complement receptor (CR1) in the maxillary sinus mucosa of normal patients and in cases of chronic sinusitis was studied with the peroxidase-antiperoxidase, avidin-biotin peroxidase and immunofluorescent methods. CR1 was localized on the ciliary surface and in the cytoplasm of the covering epithelium in both normal controls and the cases of chronic sinusitis. CR1 tended to be denser in the mucosa of chronic sinusitis than in normal mucosa. CR1-binding capacity was also studied with the immunofluorescent method, using C3b-conjugated zymosan. Although CR1 did not bind to C3b in vivo, it was found to bind to C3b in the normal maxillary mucosa when it was treated with C3b-conjugated zymosan. CR1-binding capacity could not be detected in the mucosa from cases with chronic sinusitis, indicating that CR1 was already bound to activated C3b in these cases.
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