Epidermolysis bullosa acquisita--mapping of antigenic determinants by an immunofluorescent technique.
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Biomedical subjects
Publications and source records attributed to A Bendelac.
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One case of the so-called "Stewart-Treves syndrome" (STS), appearing on a lymphoedematous arm complicating radical mastectomy for breast cancer, was characterized electronmicroscopically and immunohistologically, in order to elucidate its disputed (epithelial vs endothelial) histogenesis. Epithelial and endothelial differentiation markers used comprised: antibodies against keratin, vimentin, factor VIII-related antigen (F VIII-RA), HLA-DR antigens and the lectin Ulex europeaus agglutinin I (UEA I). At the ultrastructural level, neoplastic cells were found to contain typical Weibel-Palade bodies, whereas by immunohistological techniques they proved to be keratin-negative/vimentin+, F VIII-RA+, UEAI+, HLA-DR+. These results rule out a possible epithelial differentiation and strongly favour an endothelial one for STS.
Bullous pemphigoid is a typical autoimmune disease. It is classically treated with systemic corticosteroids alone or combined with immunosuppressants. Cyclosporine A (CyA), a new immunosuppressive agent with high activity in organs and bone marrow transplantation, could be expected to prove effective in this disease owing to its action on lymphocytes. Seven patients with bullous pemphigoid were treated with CyA in daily doses of 6 to 8 mg/kg bodyweight. Treatment was monitored by measurements of serum CyA, creatinine and liver enzyme levels. The effectiveness of treatment was assessed on clinical changes. The seven cases are dealt with individually, with a brief case-report for each of them. The only side-effects observed were reversible rises in serum creatinine levels and hypertrichosis in two cases; these are usual reactions to the drug. Hormonal assays were normal in two female patients. Concerning results, our patients fell into two groups. Among those treated with CyA alone there were two failures and two sustained satisfactory results. Treatment was successful in all patients treated with CyA during relapses under corticosteroid therapy, but two patients relapsed after CyA was discontinued. It is concluded that CyA is of no interest in the acute phase of bullous pemphigoid, that the long-term stability of the results obtained is doubtful and that this potentially nephrotoxic drug should be avoided or administered with extreme caution in elderly people, since their renal function may be at the limit of normality.
An unusual case of cutaneous nodular T cell lymphoma evolving for 4 years with massive eosinophilia and greatly increased IgE levels is discussed. Repeated histologic and immunohistologic examinations could not ascertain malignancy because tumors were composed of a granuloma-like, highly polymorphic cellular infiltrate with mature immunotype and no significant nuclear abnormalities nor epidermotropism. T cell lymphoma was evidenced by the T cell receptor beta-chain gene study, which showed a clonal rearrangement. Final histologic classification was "pleomorphic T cell lymphoma" because further biopsy samples displayed numerous lymphoid cells with pleomorphic convoluted nuclei. The T-cell receptor gene probe is a major tool for the early diagnosis of some T cell lymphomas. The case we report shares many features with the smoldering type of human T cell lymphotropic virus type I-associated Japanese endemic pleomorphic T cell lymphoma. Our virologic study confirms that in nonendemic Western countries, pleomorphic T cell lymphomas do not show evidence of a retrovirus association.
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Basement membranes were investigated in early angiomatous and late sarcomatoid stages of Kaposi's sarcoma (KS). Seven frozen skin biopsies of KS from five elderly Mediterranean people and one renal allograft recipient were labelled, using an immunoperoxidase technique, for basement membrane-specific macromolecules, laminin and type IV-collagen. Twenty-seven other frozen cutaneous lesions including haemangio and lymphangiosarcomas, benign vascular tumours, and various epithelial, melanocytic, fibrohistiocytic, fibrosarcomatous and muscular tumours were processed in the same way. In addition an ultrastructural study was performed in two cases of KS, one haemangiosarcoma and one lymphangiosarcoma. Intense labelling was observed for both type IV-collagen and laminin, which appeared closely co-distributed, in all areas of KS. Staining pattern was often regular and continuous around neoplastic vessels in early lesions of KS, as in benign vascular lesions, whereas in late nodular lesions large amounts of basement membrane components were present in intercellular spaces between densely aggregated spindle cells. In contrast, ultrastructural examination disclosed early disruption of basement membranes around neoplastic vessels, and occasional fragments of external lamina were seen at the interface between KS spindle cells and collagen. Similar results were obtained in angiosarcomas both by immunohistological and ultrastructural study. In the comparative group, laminin and type IV-collagen were present, in significantly fewer quantities and in various distinctive patterns, in epithelial, melanocytic, fibrohistiocytic and muscular tumors. This study deals with basement membrane modifications in early and late lesions of KS and provides further evidence in favour of the endothelial nature of the spindle cells of KS.
The origin of spindle-shaped cells in Kaposi's sarcoma (KS) remains controversial. Non-specific histochemical reactions, electron microscopic examinations and immunostainings using antibody against factor VIII-related antigen (F VIII-RAG) and Ulex europaeus agglutinin I (UEAI) lectin as endothelial markers have given contradictory results. Immunohistochemical techniques were applied to 7 frozen skin biopsy specimen of KS from 5 elderly Mediterranean people and 1 renal allograft recipient, and a group of 27 other frozen cutaneous tumours including haemangio and lymphangiosarcomas, benign vascular lesions and various epithelial, melanocytic, fibrohistiocytic, fibrosarcomatous and muscular tumours. Using UEAI and antibodies against F VIII-RAG, HLA-DR and vimentin, a large proportion of positive KS spindle cells was found in all cases whereas cells were negative for keratin. Among the various immunoreactivity patterns observed in this study, a unique immunohistochemical profile was demonstrated for KS, angiosarcoma and endothelial cell, which strongly supports the endothelial origin of spindle cells in KS. Whereas F VIII-RAG, HLA-DR, vimentin and UEAI were sensitive endothelial markers, only F VIII-RAG appeared specific for endothelial cells since UEAI stained 2 squamous cell carcinomas and HLA-DR and vimentin were present in various mesenchymal and melanocytic tumours.
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Malignant haemangioendothelioma of the scalp of the elderly (MHS) is a relatively rate entity. A new case of this uncommon condition, which was subjected to an ultrastructural and immunohistological study, is reported herein.
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Mycobacterium chelonei is a facultative pathogen which exists as a saprophyte in the environment and rarely produces clinical manifestations in humans. We describe a 62-year-old woman, long-term treated with low-dose steroids for severe asthma, who presented sporotrichoid-like lesions on one leg. These lesions appeared two months after a cat-scratch and were present for six months. Histological examination showed acute inflammation with polymorphonuclear infiltration without tuberculoid granuloma nor caseation necrosis. Ziehl-Neelsen stains were negative. Mycobacteria were found by direct examination and Mycobacterium chelonei chelonei was identified by culture. Treatment with isoniazid, rifampicin and ethambutol was given for one month and followed by complete resolution of the lesions, though each of these antibiotics was not effective in vitro. The literature about Mycobacterium chelonei infections is reviewed and the role of the antibiotherapy in clinical recovery is discussed.
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A case of Epithelioma cuniculatum arising on a leg ulcer in a 85 years old lady is described. 102 cases have been reported before, mainly located on the sole of the foot: this rare tumor may be recognized by the gross appearance of a slow-growing exophytic cauliflower growth, exuding foul smelling material from numerous sinuses, which eventually invades underlying tissue sometimes including bone, and which rarely metastasizes to the regional lymph nodes. Histological examination shows a well-differentiated squamous epithelial proliferation without cellular atypia, with numerous crypts and sinuses. The diagnosis is difficult as the cytological regularity does not allow malignancy to be evoked. It is the conspicuity of the invasion of the deep tissues with sufficiently large samples that makes it certain. The differential diagnosis includes keratoacanthoma, pseudo-epitheliomatous hyperplasia mainly represented by pyoderma vegetans and "classical" squamous cell carcinoma. From a nosological point of view the authors totally assimilate Epithelioma cuniculatum and Gottron's papillomatosis cutis carcinoïdes regrouped under the name of cutaneous verrucous carcinoma, emphasizing the analogy with verrucous carcinomas of mucous membrane, giant condyloma of Buschke-Lowenstein, oral florid papillomatosis.