Search PubMed⌕ Search

Biomedical subjects

P Joly

Publications and source records attributed to P Joly.

At least 127 records · Page 7Linked to original sources

Lichenoid erythrodermic bullous pemphigoid of the African patient.

BACKGROUND: Lichen planus (LP) pemphigoides is defined by the concurrence of bullous pemphigoid (BP) and LP lesions. However, other autoimmune bullous skin diseases such as paraneoplastic pemphigus or bullous lupus erythematosus may also be associated with lichenoid eruptions. OBJECTIVE: Our purpose was to describe 3 African patients with a similar form of lichenoid erythrodermic BP. METHODS: Medical records of the 3 patients were reviewed for clinical, histologic, and biologic data. The ultrastructural localization of autoantibody deposits was studied by direct and indirect immunoelectron microscopy. The antigenic specificities recognized by the patients' sera were analyzed by immunoblotting on human epidermal extracts. RESULTS: Three black African men had a severe lichenoid erythroderma associated with BP lesions involving the skin and mucosa. Histologic and direct immunofluorescence examination of skin and mucosal lesions were consistent with the diagnosis of BP associated with a lichenoid dermatitis. Immunoblot analysis of sera detected anti-BPAG1 and anti-BPAG2 antibodies and immunoelectron microscopy showed IgG deposits localized in the lamina lucida and the hemidesmosomes. The 3 patients were natives of an area near the Senegal River and had the common HLA-DR10 haplotype. CONCLUSION: The clinical and histologic features of these 3 patients were distinct from classic LP pemphigoides. Their common geographic origin and the presence of the HLA-DR10 haplotype suggest that these cases represent a particular type of lichenoid erythrodermic BP, which is probably determined by genetic factors.

Adult↗

Statistical evaluation of diagnostic and prognostic features of CD30+ cutaneous lymphoproliferative disorders: a clinicopathologic study of 65 cases.

Several clinical and histopathologic features of 65 CD30+ cutaneous lymphoproliferations were evaluated for their diagnostic value between CD30+ primary versus secondary cutaneous lymphomas and for their prognostic significance. Primary cutaneous disease, spontaneous regression, and absence of extracutaneous spreading (but not age < or =60 years) were associated with a better prognosis. Epithelial membrane antigen, BNH9, CD15 or CBF.78 antigen were expressed in all types of cutaneous lymphoproliferations. However, epithelial membrane antigen immunoreactivity was more frequently expressed in CD30+ secondary cutaneous large-cell lymphoma. Among CD30+ primary cutaneous large-cell lymphoma, CD15 expression was only seen in localized skin lesions. P53 expression was not associated with spontaneous regression, extracutaneous spreading, or survival. Nested reverse transcriptase-polymerase chain reaction allowed the detection of NPM-ALK transcripts in 10 of 26 CD30+ primary and in 3 of 11 secondary cutaneous large-cell lymphomas. The ALK protein was detected in only 1 of 50 primary and in 4 of 15 secondary cutaneous CD30+ lymphoproliferations. In CD30+ primary cutaneous lymphoproliferation, NPM-ALK transcripts might be expressed by very rare normal or tumoral cells that are undetectable by immunohistochemistry. However, the expression of either NPM-ALK transcripts or ALK-protein was not correlated with prognosis or age in CD30+ cutaneous lymphoproliferations.

Biomarkers, Tumor↗

Flow cytometry analysis of peripheral blood lymphocytes from patients with bullous pemphigoid.

BACKGROUND: Bullous pemphigoid (BP) is an autoimmune disease observed in elderly subjects in which alteration of peripheral blood lymphocyte subset repartition has been described. OBJECTIVE: To evaluate peripheral blood lymphocyte subsets from BP patients and identify immunologic changes that can be assigned either to the autoimmune disease or to the ageing process. METHODS: Peripheral blood lymphocytes from 24 patients with BP and 24 age- and sex-matched healthy individuals were analyzed by flow cytometry and these results were compared to those obtained from young adults. RESULTS: This study showed: (i) the presence of T-cell lymphopenia with a decreased number of CD8+ and gdgammadelta T lymphocytes in BP patients compared with younger adult controls; (ii) a high dispersion of the results in the BP population; (iii) the absence of a significant difference of lymphocyte subset levels between BP patients and age- and sex-matched controls. CONCLUSION: This flow cytometry study suggests that the modifications of lymphocyte subpopulation values observed in BP patients are related to immunologic changes induced by age in elderly people.

Age Factors↗

A penalized likelihood approach for arbitrarily censored and truncated data: application to age-specific incidence of dementia.

The Cos model is the model of choice when analyzing survival data presenting only right censoring and left truncation. There is a need for methods that can accommodate more complex observation schemes involving general censoring and truncation. In addition, it is important in many epidemiological applications to have a smooth estimate of the hazard function. We show that the penalized likelihood approach gives a solution to these problems. The solution of the maximum of the penalized likelihood is approximated on a basis of splines. The smoothing parameter is estimated using approximate cross-validation; confidence bands can be given. A simulation study shows that this approach gives better results than the smoothed Nelson-Aalen estimator. We apply this method to the analysis of data from a large cohort study on cerebral aging. The age-specific incidence of dementia is estimated and risk factors of dementia studied.

Age Factors↗

[Lichenoid paraneoplastic pemphigus in low-grade nodular lymphoma].

BACKGROUND: The precise diagnostic criteria for paraneoplastic pemphigus defined by Anhalt are not always present. In many cases, the clinical and histological features are misleading. CASE REPORT: We observed a case of paraneoplastic pemphigus in a 60-year-old man followed for low-grade inactive nodal lymphoma. The clinical course was long. The main features were lichenoid cutaneous and mucosal lesions without bullae. The only histology finding was basal vacuolization, lymphocyte infiltration and suspended keratinocyte necrosis without acantholysis. This clinical and pathological situation continued for one year. Just a few days prior to the patient's death, the clinical situation suddenly worsened with major bullae involving most of the skin surface and mucosal areas including the esophagus and respiratory tract. Cyclosporine and bolus injections of corticosteroids did not prevent the fatal outcome. DISCUSSION: This unusual lichenoid presentation has not been previously described. Associated skin erosions usually occur early in the disease course. The long lichenoid presentation for several months without acantholysis emphasizes the difficulties encountered in the diagnosis of paraneoplastic pemphigus. This diagnosis should be entertained in cancer patients who develop lichenoid lesions.

Biopsy↗

Early versus late coronary stenting following acute myocardial infarction: results of the STENTIM I Study (French Registry of Stenting in Acute Myocardial Infarction).

This study was undertaken to determine the feasibility and safety of coronary stenting in acute myocardial infarction (AMI). In AMI, primary percutaneous transluminal coronary angioplasty (PTCA) is accepted as the preferred method of reperfusion for patients presenting at highly experienced centres. Until recently, however, stenting has been avoided during AMI because of a potential high risk of thrombosis. This prospective observational study carried out in 20 centres and included 648 consecutive patients who underwent PTCA with stent implantation for AMI. Of these 648 patients, 269 (41.5%, Group 1) were dilated early (< 24 hr) after the onset of the symptoms (75% treated by direct PTCA) and 379 (58.5%, Group 2) were dilated between 24 hr and 14 days after AMI. Combined therapy with ticlopidin and aspirin was used after the procedure. Bailout stenting occurred more often in Group 1 than in Group 2 (17% vs. 9.5%)(P < 0.05). Angiographic successful stenting was similar in both groups of patients (96% vs. 97%). During the hospital follow-up period, stent thrombosis occurred in eight patients (3%) in Group 1 and in six patients (1.6%) in Group 2 (NS). There was 14 deaths (5.2%) in Group 1 and 11 deaths (3.9%) in Group 2 (NS). After multivariate analysis bailout stenting was identified as the sole predictor of stent thrombosis (P < 0.0001). Vascular access-site complications occurred in six patients (1%) with no difference between the two groups. This study indicates that patients who receive a coronary stent in AMI can be managed safely with antiplatelet therapy. Randomized studies are needed to determine the precise indication for coronary stenting as an adjunct to primary PTCA.

Adult↗

A complementarity-determining region peptide of an anti-desmosome autoantibody may interact with the desmosomal plaque through molecular mimicry with a cytoplasmic desmoglein 1 sequence.

The sera of patients with pemphigus, a group of autoimmune blistering skin diseases, contain autoantibodies directed against components of adhering junctions termed desmosomes. F12, a human monoclonal antibody derived from a pemphigus patient, recognizes an unknown polypeptide of the desmosomal and hemidesmosomal plaques. The third complementarity-determining region of the F12 heavy chain (VH-CDR3) was shown to share a four-amino-acid sequence (GSSG) with the intracellular domains of desmoglein 1 and bullous pemphigoid antigen 2 which interact with components of, respectively, the desmosomal and hemidesmosomal plaques. Computer modeling of F12 showed that the GSSG sequence protudes inside the antigen-combining site and thus might be involved in antigen interactions. The GSSG sequence is essential to F12 function, since a peptide containing the VH-CDR3 inhibited its binding to target antigens while VH-CDR3 peptides with specific modifications of the GSSG sequence did not. These data allow us to hypothesize that certain autoantibodies produced during the course of an autoimmune disease can behave as adhesion molecules, through the molecular mimicry of the motif involved in protein/protein adhesion, and to propose a new self-antigen binding mechanism for some autoantibodies.

Amino Acid Sequence↗

Indomethacin treatment of eighteen patients with Sweet's syndrome.

BACKGROUND: The standard treatment for Sweet's syndrome (acute febrile neutrophilic dermatosis) is oral corticosteroids. Despite a good initial response, the disease is characterized by frequent relapses. OBJECTIVE: Our purpose was to test the therapeutic effect of the nonsteroidal antiinflammatory drug indomethacin on Sweet's syndrome. METHODS: All patients with Sweet's syndrome observed during a 4-year period were given indomethacin, 150 mg/day for the first week and 100 mg/day for two additional weeks. The therapeutic response was assessed on days 4, 7, 14, 30, and 180. RESULTS: Seventeen of 18 patients had a good initial response; fever and arthralgias were markedly attenuated within 48 hours and eruptions cleared between 7 and 14 days. The remaining patient's cutaneous lesions continued to develop and were successfully treated with prednisone (1 mg/kg/day). The only side effect of indomethacin treatment was epigastric pain in two patients. No patient had a relapse after discontinuation of indomethacin (mean followup, 20.1 months). CONCLUSION: Indomethacin is a safe and effective treatment for Sweet's syndrome.

Adult↗

Taxon composition and genetic variation of water frogs in the Mid-Rhône floodplain.

Natural hemiclonal hybrid lineages of water frogs reproduce by hybridogenesis, excluding one parental genome in the germ line and mating with the coexisting same parental species. Two such sexual host-hybridogen systems occur in the Rhône valley: the L-E system in the north, the P-G system in the south. Although these hybridogenetic complexes may overlap along the Rhône river, there is no evidence for a contact zone in our samples: only Rana ridibunda and R. esculenta were identified using protein electrophoresis. Whether the absence of R. perezi reflects a more southern distribution or its exclusive occurrence in other habitats, remains to be tested. Comparison of somatic and gonadal tissues reveals that gametogenesis of R. esculenta is of the L-E type: gametes carry ridibunda genomes. R. ridibunda apparently is not native, but was introduced by humans, and the R. esculenta in our samples is probably an immigrant from nearby L-E systems.

Animals↗

Identification of a new antibody population directed against a desmosomal plaque antigen in pemphigus vulgaris and pemphigus foliaceus.

Pemphigus vulgaris and pemphigus foliaceus are characterized by autoantibodies directed against transmembrane glycoproteins of desmosomes. F12, a human monoclonal autoantibody that binds to the desmosomal plaque, recognizes a 180-190-kDa doublet when immunoblotted against bovine tongue epithelium. Because F12 was derived from the peripheral blood lymphocytes of a patient with pemphigus vulgaris, we looked for the presence of anti-180-190-kDa antibodies in pemphigus vulgaris and pemphigus foliaceus serum. By immunoblot analysis, a third of the pemphigus serum contained anti-180-190-kDa antibodies that belonged to IgG subclass 1 or 3, unlike those that recognized desmogleins 1 and 3 (IgG4). By immunoelectron microscopy analysis on human oral mucosa and human skin with mAb to human IgG3, pemphigus serum containing anti-180-190 kDa antibodies recognized desmosomal plaques. The presence of antibodies with F12 properties in pemphigus serum was further demonstrated by a rabbit anti-F12 idiotype antiserum that allowed detection of F12 idiotype in serum with anti-180-190-kDa antibodies. These results indicate that some pemphigus vulgaris and pemphigus foliaceus serums contain antibodies that react with both intra- and extracellular structures of desmosomes and further demonstrate the heterogeneity of the autoimmune response in both types of pemphigus.

Antibodies, Monoclonal↗

Relationship between the in vivo localization and the immunoblotting pattern of anti-basement membrane zone antibodies in patients with bullous pemphigoid.

OBJECTIVE: To compare the localization of anti-basement membrane zone (BMZ) antibodies bound in vivo with the antigenic specificities of circulating anti-BMZ antibodies in patients with bullous pemphigoid (BP). DESIGN: Comparison of the results of an examination of the skin specimens of the patients using direct immunoelectron microscopy and direct immunofluorescence on 1-mol/L sodium chloride-split skin with the results of an analysis of the corresponding serum samples using the immunoblot technique. SETTING: Immunodermatology department in a teaching hospital. PATIENTS: Thirty-six patients with typical BP and circulating anti-BMZ antibodies. RESULTS: Serum samples from 22 patients with BP indicated only BP antigen 1 in the results of immunoblot analysis. Using direct immunofluorescence, an analysis of the peribullous skin samples obtained from these 22 patients showed deposits of IgG exclusively located along the epidermal side of sodium chloride-split skin; the results of direct immunoelectron microscopic examination showed deposits of IgG located on the intracellular portion of hemidesmosomes in 18 (82%) of these 22 specimens, whereas 4 biopsy specimens had linear IgG deposits located both intracellularly and extracellularly along the keratinocyte plasma membrane. The results of immunoblot analysis of the serum samples from 5 patients with BP indicated BP antigen 2 alone; the results of direct immunoelectron microscopic examination of peribullous skin samples from these 5 patients showed linear intracellular and extracellular deposits along the keratinocyte membrane, corresponding to an epidermal fluorescence labeling pattern of peribullous sodium chloride-split skin in 2 patients and a combined (dermal and epidermal) pattern in 3 patients. CONCLUSION: The 2 different patterns of reactivity of anti-BMZ antibody deposits bound in vivo closely corresponded to the antigenic specificities indicated in the corresponding serum samples of the patients. These results are in accordance with those previously obtained in vitro and argue for identical binding profiles of circulating antibodies that are bound in vivo in BP.

Autoantibodies↗

Autoantibody formation against a 190-kDa antigen of the desmosomal plaque in pemphigus foliaceus.

We report changes in the antigen recognition pattern of sera from two pemphigus foliaceus patients with a long-term follow-up. The patients' sera were analysed by immunoblotting using different antigenic sources: cultured human keratinocytes, bovine tongue epithelium and a recombinant protein corresponding to the C-terminal end of the 230-kDa bullous pemphigoid antigen. While initial serum samples reacted exclusively with the 160-kDa desmoglein 1, the later sera reacted both with desmoglein 1 and a 190-kDa antigen immunolocalized to the desmosomal plaque, previously demonstrated to be recognized by sera of some patients with paraneoplastic pemphigus. IgG subclass analysis further showed that antidesmoglein 1 antibodies were of IgG1 and/or IgG4 subclasses, while anti-190-kDa antibodies were IgG3. The patients were free of malignancy.

Aged↗

[Aneurysm of superficial temporal artery in angiolymphoid hyperplasia lesions].

INTRODUCTION: Subcutaneous angiolymphoid hyperplasia with eosinophilia is a disease which usually affects small vessels of neck and facial area. CASE REPORT: A 44 year-old with cutaneous lesions corresponding to angiolymphoid hyperplasia with eosinophilia is reported. The lesions were located near the temporal region. This case was particular because of the development of an anevrism of the superficial temporal artery. DISCUSSION: Involvement of the superficial temporal artery by angiolymphoid hyperplasia with eosinophilia has been reported in only one case which simulated a Horton disease because of an occlusion of the temporal artery. The development of an anevrism of this artery in a patient with angiolymphoid hyperplasia with eosinophilia is a very unusual presentation of the disease which has not been previously reported.

Adult↗