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P Joly

Publications and source records attributed to P Joly.

At least 73 records · Page 4Linked to original sources
PubMed2001

Prognostic factors in primary cutaneous large B-cell lymphomas: a European multicenter study.

PURPOSE: Most primary cutaneous B-cell lymphomas have an excellent prognosis. However, primary cutaneous large B-cell lymphomas (PCLBCLs) of the leg have been recognized as a distinct entity with a poorer prognosis in the European Organization for Research and Treatment of Cancer (EORTC) classification. This distinction on the basis of site has been debated. Our aim was to identify independent prognostic factors in a large European multicenter series of PCLBCL. PATIENTS AND METHODS: The clinical and histologic data of 145 patients with PCLBCL were evaluated. According to the EORTC classification, 48 patients had a PCLBCL of the leg and 97 had a primary cutaneous follicle center-cell lymphoma (PCFCCL). Data from both groups were compared. Univariate and multivariate analyses of specific survival were performed using a Cox proportional hazards model. RESULTS: Compared with PCFCCL, PCLBCL-leg were characterized by an older age of onset, a more recent history of skin lesions, a more frequent predominance of tumor cells with round nuclei and positive bcl-2 staining, and a poorer 5-year disease-specific survival rate (52% v 94%; P <.0001). Univariate survival analysis in the entire study group showed that older age, a more recent onset of skin lesions, the location on the leg, multiple skin lesions, and the round-cell morphology were significantly related to death. In multivariate analysis, the round-cell morphology (P <.0001), the location on the leg (P =.002), and multiple skin lesions (P =.01) remained independent prognostic factors. The round-cell morphology was an adverse prognostic factor both in PCLBCL-leg and in PCFCCL, whereas multiple skin lesions were associated with a poor prognosis only in patients with PCLBCL-leg. CONCLUSION: With site, morphology, and number of tumors taken into account, guidelines for the management of PCLBCL are presented.

Adult↗
PubMed2001

Sporadic multiple primary melanoma cases: CDKN2A germline mutations with a founder effect.

Multiple primary cancers are one of the hallmarks of inherited predisposition. Outside the familial context, multiple primary tumors could be related either to germline de novo mutations or to low-penetrance mutations, in predisposing genes. We selected 100 patients who displayed multiple primary melanoma (MPM) without any known melanoma cases recorded within their families and looked for germline mutations in the two melanoma-predisposing genes identified to date, CDKN2A and CDK4 exon 2. Nine patients (9%) had germline mutations in CDKN2A, whereas none carried germline mutations in exon 2 of CDK4. Seven cases displayed a recurrent missense mutation, G101W, already described in more than 20 melanoma-prone families; one case carried a missense mutation never reported to date (P114S), and the last case was a carrier of a 6 bp insertion at nucleotide 57 resulting in a duplication of codons 18 and 19. To ascertain whether the G101W was a mutational hot spot for de novo mutations or a common founder mutation, we genotyped eight microsatellite markers flanking the CDKN2A gene. After allowing for recombination over time, haplotype sharing provided evidence for an original G101W mutation common to 6 out of 7 sporadic MPM cases. Therefore, it can be concluded that de novo germline CDKN2A mutations associated with MPM are rare.

Adult↗
PubMed2001

Viral load as a primary outcome in human immunodeficiency virus trials: a review of statistical analysis methods.

In Human Immunodeficiency Virus infection, several statistical methods are available to analyze viral load (HIV-1 RNA) used as a surrogate outcome in trials of antiretroviral treatments. We compared the most frequently used methods and applied them to one of these trials, where HIV-1 RNA was measured using two lower limits of detection. Methods were reviewed for different properties dealing with validity, interpretation, and handling. Compared to change of HIV-1 RNA at the end of follow-up or HIV-1 RNA area-under-the-curve during follow-up minus baseline, the most attractive methods appeared to be HIV-1 RNA undetectability, HIV-1 RNA reduction at the end of follow-up with censoring adjustment, and mixed linear model on HIV-1 RNA.

Area Under Curve↗
PubMed2001

A polymorphic variant of the gene coding desmoglein 1, the target autoantigen of pemphigus foliaceus, is associated with the disease.

Two polymorphic markers were identified on the desmoglein 1 gene which encodes the autoantigen targeted by pathogenic antibodies in pemphigus foliaceus (PF), a cutaneous autoimmune blistering disease. The first marker, made of a variant haplotype of five mis-sense mutations located on the part of the gene encoding the fourth and fifth extracellular domains of the protein, is not associated with the disease. The second marker consists of a single silent T to C transition at position 809 and was found to be significantly more frequent (P = 0.015) in Caucasian PF patients (n = 36) than in controls (n = 98). Thus, pemphigus foliaceus constitutes another example of autoimmune disease in which the autoantigen polymorphism contributes to disease susceptibility.

Amino Acid Sequence↗
PubMed2001

Neutrophilic myositis as an extracutaneous manifestation of neutrophilic dermatosis.

Neutrophilic dermatoses are characterized histologically by a sterile infiltration of neutrophils throughout the dermis. The condition may affect various internal organs, notably the lungs, the digestive tract, and the joints, whereas muscle impairment is considered to be extremely rare. We describe the case of a patient with pyoderma gangrenosum in whom severe sterile neutrophilic myositis developed as the first manifestation of an acute myelogenous leukemia.

Aged↗
PubMed2001

IGHV3-associated restriction fragment length polymorphisms confer susceptibility to bullous pemphigoid.

OBJECTIVE: To determine whether the immunoglobulin heavy chain genes contribute to the occurrence of bullous pemphigoid (BP), polymorphisms of both the immunoglobulin constant IGHC and variable IGHV groups were studied in 100 Caucasian BP patients and 143 ethnically matched healthy individuals. METHODS: To analyze the restriction fragment length polymorphism (RFLP) of the IGHG constant locus, a genomic immunoglobulin gamma 3 probe which detects polymorphisms in the gamma constant genes was hybridized to BstEII- or BamHI/SacI-digested germline DNA, while IGHV3 subgroup polymorphism was analyzed by hybridizing a cloned VH3 probe to EcoRI-digested DNA. RESULTS: No difference in the frequencies of the genotypes defined by the constant probe was observed between patients and controls. Analysis of the RFLP obtained with the VH3 probe showed that within the range of 4.5 and 1.5 kb, the observed band pattern was composed of 8 monomorphic and 7 polymorphic bands. Among the latter, 4 allowed to define 10 different restriction patterns. One pattern was shown to be significantly less frequent in patients than in controls. CONCLUSION: IGHV3 polymorphism might be a factor conferring susceptibility to BP.

Deoxyribonucleases, Type II Site-Specific↗
PubMed2001

[Histological and clinical forms of the eosinophilic cellulitis].

BACKGROUND: Wells' syndrome is characterized by clinical features of cellulitis and a histological picture of eosinophilic infiltrate of the dermis with some "flame" figures. PATIENTS AND METHODS: The clinical and histological features of nine patients with Wells' syndrome seen from 1988 to 1998 were retrospectively reviewed. RESULTS: The clinical features of the nine patients (five men and four women) were urticaria (n=1), cellulitis (n=2), annular plaques (n=3), vesiculo-bullous lesions (n=2) and edema of the face with nodules of the conjunctiva (n=1). Histological examination of skin biopsies showed an eosinophilic infiltrate of the dermis associated with some "flame" figures in all cases. The infiltrate was located in the superficial or deep dermis in accordance with the different clinical features. One patient developed a non Hodgkin lymphoma and presented successively: a Wells' syndrome, a leucocytoklastic vasculitis and a Sweet's syndrome. Numerous treatment were used: topical corticosteroids, H1-antihistamines, dapsone and systemic corticosteroids. Two patients relapsed after treatment withdrawal. DISCUSSION: This study demonstrated a wide polymorphism of the clinical and histological features of Wells' syndrome. The clinical features seem to depend on the location of the dermal infiltrate, suggesting the existence of a spectrum of eosinophilic dermatoses, like in neutrophilic dermatoses. The successive occurrence of vasculitis, Wells' syndrome and Sweet'syndrome in a patient suggests an overlap between these diseases. Systemic corticosteroids are the most effective treatment, but may lead to a corticosteroid dependence.

Adolescent↗
PubMed2001

[Detection of clonal T-cell receptor gamma gene rearrangement with the use of PCR-DGGE for diagnosis of erythroderma].

BACKGROUND: It is often difficult to establish the etiological diagnosis of erythroderma because clinical findings and immunohistology cannot always distinguish between lymphomatous erythroderma and inflammatory erythroderma. The purpose of this work was to assess the contribution of PCR-DGGE for detecting clonal T-cell receptor gamma gene rearrangement to the etiological diagnosis of erythroderma. PATIENTS AND METHODS: The following inclusion criteria were used: patient with erythroderma; skin biopsy for histologic study, immunophenotyping and molecular biology; minimal follow-up of 12 months after initial diagnosis. Thirty patients were included from May 1, 1995 to November 30, 1998. Histology slides were reread by one of the authors blinded to other data who classed them in three categories: probable lymphoma, probable inflammatory disease, uncertain diagnosis. Molecular data were also analyzed in the same blinded manner. Immunohistology diagnosis was compared with the molecular data and the final diagnosis retained from clinical, histological and molecular findings as well as the disease course to last follow-up (November 1, 1999) after a mean 12 +/- 18 months follow-up. RESULTS: Eight biopsies were classed as probable lymphomas; a T-cell clonal rearrangement of the TCR genes was detected in 7/8 cases. The one sample with no detectable T clone was a drug-induced Sézary pseudolymphoma. The histologial classification identified 16 cases of probable inflammatory disease; no clonal rearrangement of the TCR genes was found in these cases. One of these patients had fungoid mycosis treated with caryolysin for three months and developed treatment intolerance at the time of the skin biopsy. For six biopsies the histological diagnosis was "uncertain"; a clonal rearrangement of the TCR genes was found in 2/3 of the fungoid mycosis cases and in none of the three cases of toxic dermal reactions. DISCUSSION: This study demonstrated the contribution of genotypic analysis with PCR-DGGE to the diagnosis of erythroderma. Monoclonal TCR gene rearrangement was detected in 9/11 (82 p. 100) of the patients with lymphoma and in 0/19 of the patients with an inflammatory dermatosis. The etiological diagnosis of erythroderma is an excellent indication for molecular stud of skin biopsies with PCR-DGGE.

Dermatitis, Exfoliative↗
PubMed2001

[Hydroa vacciniforme: dietary fish oil].

BACKGROUND: Hydroa vaccinniforme is a highly uncommon photodermatosis acquired in childhood. The clinical course is dominated by the risk of varioliform scars. Numerous treatments have been proposed with variable efficacy. One recent open study suggested dietary fish oil could be useful. We report a case of hydroa vacciniforme treated successfully with dietary fish oil (Maxepa(R)). CASE REPORT: A 15-year-old girl consulted in May 1988 for vesiculobullous lesions typical of hydroa vacciniforme in photo-exposed areas. Anti-malaria drugs and photoprotection had been used for several years without success. Maxepa(R) was introduced in June 1998 and was followed by regression of the lesions within a few weeks despite the summer season. The treatment was interrupted at the patient's request due to fetid breath. Reintroduction of Maxepa(R) in April 1999 after an episode of recurrent lesions, again led to total resolution of the lesions within three weeks. DISCUSSION: Hydroa vacciniforme is an exceptional photodermatosis of uncertain etiology. It may possibly be related to an abnormal sensitivity to ultraviolet A. Fish oil rich in 3-omega polyunsaturated fatty acids would reduce the local inflammation triggered by sun exposure. Recent studies have demonstrated that dietary fish oil can increase the level of 3-omega polyunsaturated fatty acids in the epidermis and reduce the level of prostaglandins in the skin. Our case would appear to confirm the contribution of dietary fish oil to treatment despite the poor tolerance due to fetid breath.

Adolescent↗
PubMed2001

[Cutaneous aseptic abscesses, manifestations of neutrophilic diseases].

BACKGROUND: Neutrophilic skin disease includes several entities: Sweet syndrome, pyoderma gangrenosum, erythema elevatum diutium, Sneddon-Wilkinson sub-keratous pustulosis, and neutrophilic eccrine hidradenitis. We report two cases of aseptic abscesses which correspond to the deepest anatomoclinical form of neutrophilic dermatosis. CASE REPORTS: A 28-year-old man was hospitalized for fever and abdominal pain with bloody diarrhea in relation with Crohn's disease. The patient also presented two skin abscesses on the lower limbs. Bacteriology specimens were negative. The histology specimen of a skin lesion revealed neutrophil infiltration of the hypodermis without granulomatosis. Systemic corticosteroid therapy was given and rapidly led to resolution of the inflammatory bowel disease and the skin lesions. The patient developed inflammatory spondylarthropathy several months later. The second patient was a 36-year-old woman with a history of splenomegaly with asceptic abscesses. She was admitted for abdominal pain with non-bloody diarrhea, fever and multiple joint pain related to spondylarthropathy. She developed several simultaneous abscessed nodules on the legs. Biopsy revealed neutrophil infiltration of the hypodermis. The diagnosis of neutrophilic disease with aseptic cutaneous and visceral abscesses was retained. Nonsteroidal antiinflammatory drugs and dapsone were given leading to regression of the skin lesions and the abdominal and joint pain. DISCUSSION: Aseptic skin abscesses result from a deep localization of neutrophilic disease. They suggest the presence of inflammatory bowel disease, spondylarthropathy or other aseptic visceral localizations.

Abscess↗
PubMed2001

[Paraneoplastic pemphigus: a pustular form during chronic lymphoid leukemia].

BACKGROUND: Paraneoplastic pemphigus is an autoimmune disease of the skin and mucosa described in 1990. The condition is generally associated with lymphoma or chronic lymphoid leukemia. Lesions are often misleading, masquerading as polymorphous erythema or lichen. We report a case of paraneoplastic pemphigus with pustulous skin lesions. CASE REPORT: A 52-year-old man developed over a few weeks time erosive lesions of the oral cavity and lips associated with papulous skin lesions. Secondarily, large-sized pustules, sometimes a hypopion, were observed associated with bullae. The diagnosis of paraneoplastic pemphigus was confirmed by direct immunofluorescence that evidenced IgG deposits within the keratinocytes and along the basal membrane and by indirect immunofluorescence on rat bladder that evidenced circulating antibodies. This paraneoplastic pemphigus was the inaugural sign of chronic lymphoid leukemia. DISCUSSION: Skin lesions described in paraneoplastic pemphigus include: erosion, vesicles, bullae, and psoriasiform, lichen-like, plate-like or vegetative formations. To our knowledge, this is the first report of a pustulous form; clinically similar to Hallopeau pustulous pemphigus.

Anti-Inflammatory Agents↗
PubMed2001

[Mouth ulcers in patients receiving tacrolimus].

INTRODUCTION: The buccal side effects of immunodepressors are well defined with cyclosporine and certain antimitotic agents. We report a case of buccal ulcerations in a patient treated with a new immunosuppressive macrolide: tacrolimus (Prograf). OBSERVATION: A 53 year-old woman presenting a severe cardio-myopathy, underwent heart transplantation in May 1997. Tacrolimus was introduced in October 1997 after 3 episodes of acute reject. Eight months after tacrolimus, painful apthoid buccal ulcerations appeared. Biopsy of the buccal mucosa and other biological examinations revealed no particular etiology. Since tacrolimus could not be stopped, treatment with thalidomide was initiated. It was suspended on two occasions due to adverse events. The buccal ulcerations relapsed rapidly. The intrinsic imputability of tacrolimus in the occurrence of these lesions was noted "l2" ("plausible"). DISCUSSION: Several arguments suggest that these buccal ulcerations may result from the toxicity of tacrolimus: 1) absence of past history of apthae; 2) anatomo-clinical aspect of the lesion differing from that of common apthae, but similar to the ulcerations observed with nicorandil; 3) delay in occurrence of analogous ulcerations compared with that observed with methotrexate or nicorandil; 4) absence of another etiology; 5) relapse of ulcerations on two occasions after suspension of thalidomide, whilst tacrolimus was continued.

Biopsy↗
PubMed2000

Neoteny and progenesis as two heterochronic processes involved in paedomorphosis in Triturus alpestris (Amphibia: Caudata).

Current theories on the evolution of paedomorphosis suppose that several ontogenetic pathways have appeared according to different selective pressures. The aim of this study was to find out whether two distinct processes can lead to paedomorphosis in the Alpine newt, Triturus alpestris. In this respect, we compared age structures of paedomorphic and metamorphic individuals in two newt populations where the two forms lived syntopically. Whereas paedomorphosis resulted in a slower rate of somatic development in one population, it resulted in an acceleration of sexual maturation in the other population. These processes correspond to neoteny and progenesis, respectively. These results suggest that phenotypic plasticity can result from contrasted ontogenetic pathways between two populations of the same species. They give support to models that consider gonadic development as the target of selection under different environmental pressures.

Animals↗
PubMed2000

Transformation of mycosis fungoides: clinicopathological and prognostic features of 45 cases. French Study Group of Cutaneious Lymphomas.

The course of mycosis fungoides (MF) is indolent except when transformation to a large T-cell lymphoma occurs. The diagnosis of transformed MF (T-MF) relies on the presence of more than 25% of large cells on biopsy of an MF lesion. We analyzed 45 patients with T-MF recorded by the French Study Group on Cutaneous Lymphomas to better determine clinicopathological features of MF transformation and to analyze their impact on prognosis. Median time from diagnosis of MF to transformation was 6.5 years. Extracutaneous progression was present in 20 patients. Mean survival from transformation to death was 22 months. In univariate analysis, only an extracutaneous progression was associated with a worse prognosis (5-year actuarial survival: 7.8% versus 32%). Neither sex, age, clinical and skin disease stage at transformation, transformation speed, nor percentage of large cells or CD30 expression (14 of 45) had a prognostic value. When performing multivariate analysis, age (at least 60 years), and extracutaneous spreading were found to be associated with a poor prognosis. There was no difference between survival curves of patients with T-MF and with pleomorphic large T-cell CD30- lymphomas. The main diagnostic pitfall was "histiocytic-rich" MF, requiring CD68 staining for the diagnosis of T-MF. Out of 45 patients, 6 presented an histologic transformation before clinical progression, suggesting that an early histopathological diagnosis may be performed by histological follow-up. The prognostic value of such early histopathological diagnosis must be confirmed by prospective studies.

Adult↗
PubMed2000

Bullous pemphigoid and amyotrophic lateral sclerosis: a new clue for understanding the bullous disease?

BACKGROUND: Bullous pemphigoid (BP) occurs in many patients with multiple sclerosis. Isolated cases of BP in patients with other neurological disorders further support a pathogenic association between cutaneous and neurological diseases. Any description of BP in patients with amyotrophic lateral sclerosis is lacking. OBSERVATIONS: We studied a French population of 168 patients with typical amyotrophic lateral sclerosis. Among these, 3 had clinical and histological features of BP. The mean age of the patients was 54 years. None was known to have autoimmune disorders. Results of immunoblot analysis disclosed both anti-BP antigen 1 and anti-BP antigen 2 antibodies. CONCLUSIONS: Bullous pemphigoid seems to be unexpectedly associated with amyotrophic lateral sclerosis. On the basis of the cases presented herein, we discuss the epidemiological significance of the association and the possible interrelation between BP antigen 1 and neurofilaments in the pathogenesis of both disorders.

Aged↗
PubMed2000

Cutaneous involvement in patients with angioimmunoblastic lymphadenopathy with dysproteinemia: a clinical, immunohistological, and molecular analysis.

OBJECTIVE: To determine whether cutaneous involvement in patients with angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) is related to a clonal T-cell proliferation. DESIGN: Retrospective study. SETTING: University hospitals. PATIENTS: Ten patients with AILD and cutaneous involvement. MAIN OUTCOME MEASURE: The T-cell receptor-gamma (TCRG)gene rearrangement was studied with the use of polymerase chain reaction and denaturing gradient gel electrophoresis in blood, nodal, and skin samples. Skin and nodal samples were investigated also for the presence of Epstein-Barr virus (EBV) RNA by in situ hybridization. RESULTS: A transient morbilliform eruption of the trunk was seen most often. Other cutaneous features were infiltrated plaques and purpuric or urticarial lesions. A clonal TCRG gene rearrangement was detected in 7 skin samples, corresponding to a maculopapular eruption with a histological pattern of nonspecific mild lymphoid dermal infiltrate in 6 patients, and to erythematous plaques with histological findings of typical cutaneous lymphoma in 1 patient. In the 5 patients in whom a TCRG gene rearrangement was evidenced in skin and lymph node samples, identical clones were detected in both. Five patients died by the end of the study, with a mean survival of 33.2 months. Four of these 5 patients had a clonal infiltrate in skin and lymph nodes. The EBV RNA was detected in only 1 of 10 skin biopsy specimens and in 5 of 8 lymph nodes tested. CONCLUSIONS: Cutaneous involvement is often related to a clonal T-cell proliferation in AILD, even when clinical and histological features are nonspecific. Cutaneous infiltrate seems to be clonally related to the nodal T-cell proliferation. The role of EBV infection in skin lesions was not evidenced.

Adult↗
PubMed2000

What, if anything, does visual asymmetry in fallow deer antlers reveal?

Fluctuating asymmetry (FA), small directionally random deviations from perfect bilateral symmetry, is thought to reflect individual quality. Furthermore, it has been suggested that FA in secondary sexual characters can be used to assess mate or opponent quality during inter- or intrasexual competition. Studies on fallow deer, Dama dama, have suggested that FA in antlers reflects individual dominance, or the existence of a directional asymmetry (DA) with right antlers being consistently more developed than left antlers. To test these conflicting hypotheses, we analysed relationships between age, dominance and asymmetry in the number of antler points on mature fallow deer males during four rutting seasons in a single population. Age and dominance were only weakly correlated. The number of antler points displayed a pattern of DA (more points on the right than on the left side) that increased with age. Although dominance tended to increase with the total number of antler points, there was no relationship between the level of DA and dominance. These results failed to support the hypothesis that antler asymmetry visually reveals individual quality in fallow deer. Copyright 2000 The Association for the Study of Animal Behaviour.

Journal Article↗