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F Caux

Publications and source records attributed to F Caux.

34 records · Page 2Linked to original sources

Binding of autoantibodies is not restricted to desmosomes in pemphigus vulgaris: comparison of 14 cases of pemphigus vulgaris and 10 cases of pemphigus foliaceus studied by western immunoblot and immunoelectron microscopy.

Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are autoimmune blistering diseases characterized by a loss of cell-cell adhesion and by autoantibodies directed against epidermal cadherins. PF antigen has been established as desmoglein I which is located strictly on the desmosome, whereas the precise ultrastructural localization of PV antigen remains unclear and controversial to date. To further investigate this question, we compared the location of immune deposits in 14 patients with PV and 10 patients with PF by both direct and indirect immunoelectron microscopy (IEM). Inclusion criteria were based upon clinical features, histological level of cleavage and characterization of circulating antibodies by Western blot on epithelial bovine tongue extracts. IEM was performed on unfixed 0.7-mm slices of skin for the direct technique or on normal skin for the indirect technique using peroxidase labelling. In PF, by both direct and indirect IEM, immune deposits were located on the extracellular part of desmosomes (desmoglea) in all the samples studied. In PV, by both direct and indirect IEM, deposits were situated on the desmoglea and along large portions of the keratinocyte membrane without desmosomal structures in 15 of the 18 samples studied and only on the desmoglea in 3 samples. These results suggest that, in contrast to PF, the target antigen in PV is not always restricted to desmosomes. As various types of adherens junctions have been reported to mediate cell adhesion in the epidermis, the PV antigen could be a component of desmosomes and of other focal adhesions.

Adult↗

Localized epidermolysis bullosa acquisita of the esophagus in a patient with Crohn's disease.

Epidermolysis bullosa acquisita is a rare autoimmune subepidermal bullous disease that affects both the skin and mucosae and is frequently associated with Crohn's disease. We report the case of a 27-yr-old man with Crohn's disease who presented with localized epidermolysis bullosa acquisita of the esophagus, without any other mucosal or cutaneous lesions. The patient was successfully treated with sulfasalazine.

Adult↗

Hyperkeratotic spicules and monoclonal gammopathy.

Hyperkeratotic spicules are a rare disorder that has been associated with different types of malignancies. An association with multiple myeloma raises the question of a relation between the monoclonal component and skin manifestations. We describe hyperkeratotic spicules in a 61-year-old man with a monoclonal gammopathy of undetermined significance. Immunofluorescence and immunoelectron microscopic findings demonstrated the deposition of the monoclonal immunoglobulin at the dermoepidermal junction. Our findings support previous reports that the association between hyperkeratotic spicules and monoclonal gammopathy is not fortuitous and may be related to particular properties of the monoclonal immunoglobulin.

Humans↗

[Ultrastructural localisation of pemphigus vulgaris and pemphigus foliaceus antigens by indirect immunoelectron microscopy. Apropos of 7 cases].

INTRODUCTION: Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are autoimmune blistering diseases characterized by loss of cell-cell adhesion and by autoantibodies directed against epidermal cadherins. The ultrastructural localization of PV antigen remains controversial, whereas the location of PF antigen seems to be established. The use of different techniques could explain these various data. To investigate this matter, indirect immunoelectron microscopy (IEM) and Western blot analysis on bovine tongue epithelium were used. MATERIAL AND METHOD: Serum samples from patients with PF(3), PV (4) and control samples from healthy patients (2) were analysed in this study. The inclusion criteria were based upon characteristic clinical features, level of epidermal cleavage on histological preparations and presence of circulating anti-epithelial cell surface antibodies. Indirect IME was performed on normal human skin. Peroxidase labelling was used. Serum samples were also analysed by western immunoblotting on bovine tongue epithelium. RESULTS: Indirect IEM examination of PV sera showed immune deposits located both on desmosomal and extra-desmosomal areas, whereas in PF, IgG deposits were strictly localized on desmosomal structures. By Western blot analysis, PV sera recognized a 130 kDa polypeptide and PF sera a 150 kDa polypeptide. DISCUSSION: Indirect IEM on normal human skin using peroxidase labelling was used because of the best antigenic conservation obtained. Our results suggest that PV antigen could exist both on desmosomal junctions and adherens junctions, whereas PF antigen (desmoglein I) is restricted to desmosome.

Adult↗

Increased serum and blister fluid levels of creatine kinase in patients with toxic epidermal necrolysis.

Keratinocytes have recently been reported to contain creatine kinase (CK) of brain-type isoenzyme. The aim of this study was to investigate whether necrosis of keratinocytes induced raised CK levels in toxic epidermal necrolysis (TEN). The serum and blister fluid levels of creatine kinase and its isoenzymes [muscular-type (MM), brain-type (BB), myocardial-type (MB)] were measured in 40 patients with TEN, 10 patients with other bullous dermatoses, and in suction blisters in five controls. The mean serum CK was significantly higher in TEN patients than in patients with other bullous dermatoses (mean +/- SD: 480 +/- 535 U/l vs. 107 +/- 44 U/l, P < 0.05). The MM-isoenzyme was predominant (94%). A positive correlation was found between the level of the serum CK and the percentage of body surface area (BSA) involved (r = 0.49, P < 0.001). The mean blister CK was significantly higher in TEN patients than in patients with other bullous dermatoses or controls (mean +/- SD: 728 +/- 437 U/l vs. 310 +/- 244 U/l and 268 +/- 194 U/l, respectively, P < 0.02). The isoenzyme distribution of blister CK in TEN patients was: 76.8% MM, 18.1% MB and 5% BB. Although a significant part of blister CK comigrating with CK-MB, after preincubation with protein A-Sepharose, appeared to be CK-BB/IgG complex, the CK-BB fraction constituted less than 25% of blister CK. Therefore, the CK present in increased amounts in serum and blister fluid in TEN was not directly produced by keratinocytes.

Adolescent↗

Erythema gyratum repens. A case studied with immunofluorescence, immunoelectron microscopy and immunohistochemistry.

We report a patient with erythema gyratum repens (EGR), in whom a bronchial carcinoma was found. Direct immunofluorescence revealed granular deposits of immunoglobulins at the basement membrane zone (BMZ) in the skin, and in the lung tumour. Direct immunoelectron microscopy showed that the immune deposits were localized just beneath the lamina densa. Indirect immunofluorescence revealed circulating anti-BMZ antibodies. Immunohistochemical staining, using anti-transforming growth factor-beta, anti-epidermal growth factor receptor, anti-vimentin and anti-alpha-actin, was found to be more intense in the lesional skin and the lung tumour than in normal tissues. Possible mechanisms in the pathogenesis of EGR are discussed.

Autoantibodies↗

[Abnormal fibrinolysis in Degos disease. A study of 3 cases].

INTRODUCTION: Degos' disease is a rare dermatosis characterized by papular lesions with a porcelain-white central atrophy and histopathological aspect of wedge-shaped infarction necrosis and an endovasculitis in the dermis. Its pathogenesis is unknown but many abnormalities of haemostasis have been reported. PATIENTS AND METHODS: Platelets functions, coagulation and fibrinolysis were estimated in three patients with Degos' disease. For one patient, direct immunoelectron microscopy using an antibody to von Willebrand factor was performed on lesional skin. RESULTS: In all the patients, prolonged euglobulin lysis time, increased plasminogen activator (PA) and plasminogen activator inhibitor (PAI) activities before and after a venous occlusion test were detected and indicated an inhibition of fibrinolysis. Electron microscopy demonstrated in one case an increased number of Weibel-Palade bodies and a raised staining of von Willebrand factor in endothelial cells. Tests for coagulation and circulating anticoagulant were normal. Results of platelets adhesion showed decrease of adhesion in one case and increased adhesion in another. Platelets aggregation studies were normal in two cases and showed hyperactive spontaneous and induced aggregation in one case. CONCLUSION: We showed an inhibition of fibrinolysis in three patients with Degos' disease. These abnormalities could induce a prethrombotic state. The release of PA and PAI from the endothelial cells into the blood stream and the modifications observed with electron microscopy may signify a primary lesion of endothelial cell of still unknown origin.

Atrophy↗

Accumulation of heparan sulfate in the culture of human melanoma cells with different metastatic ability.

Glycosaminoglycans were metabolically labeled in subconfluent cultures of highly metastatic 7Gp122 and poorly metastatic IC8 variants and of the low metastatic parental M4Be human melanoma cell line. Proteoglycans were separated by DEAE Trisacryl chromatography from the culture medium, from the heparin extract of the cell layer and from the heparin-extracted cell residue lyzed with detergents. Glycosaminoglycans were released from the proteoglycans by reductive alkaline hydrolysis and heparan sulfate (HS) was detected by deaminative cleavage with nitrous acid. Expressed on cell protein basis, the labeled HS content in the medium and in the cell layer decreased with increasing metastatic ability. The extraction of HS with heparin from the 7Gp122 cells indicated that this variant was enriched in (polypeptide bound) HS non inserted into the plasma membrane, compared with the low metastatic IC8 and M4Be cells. The HS fraction in heparin extract and in the heparin-extracted cell residue exhibited molecular mass heterogeneity on gel permeation chromatography and it contained HS fragments. Scission with nitrous acid followed by molecular sieve chromatography of the degradation products indicated that the tetra- and disaccharide repeats separated by the N-sulfated glucosamine residue were present in about equal amounts and constituted 60% of the HS chains in the IC8 and M4Be cells. HS from 7Gp122, IC8 and M4Be cells did not bind antithrombin III with high affinity but it was capable of binding bFGF in in vitro assay.

Antithrombin III↗

Fusarium solani cutaneous infection in a neutropenic patient.

A 47-year-old woman with acute myeloblastic leukemia developed disseminated papules and pustules after chemotherapy. A skin biopsy specimen revealed fungal vasculitis and grew Fusarium solani. The patient died despite treatment with amphotericin B. The isolate was resistant to amphotericin B, imidazole and 5-fluocytosine. Fusarium should be recognized as a potential cause of deep fungal infection in immunocompromised patients.

Dermatomycoses↗

[Neonatal lupus].

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Age Factors↗