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B Vergier

Publications and source records attributed to B Vergier.

At least 19 recordsLinked to original sources
PubMed2006

[Childhood-onset multifocal atrophic dermatofibrosarcoma].

BACKGROUND: Atrophic dermatofibrosarcoma is a rare clinical variant of dermatofibrosarcoma protuberans (or Darier-Ferrand tumor) preferentially observed in childhood and early adulthood. OBSERVATION: We report a case of multifocal atrophic dermatofibrosarcoma protuberans of childhood onset only diagnosed when the patient was 29 years old. The clinical presentation was an asymptomatic macular brown plaque on the right thigh measuring 10 cm. Initially, because of the large size of the lesion, treatment consisted of limited surgical excision. DISCUSSION: Diagnosis of the atrophic variant of dermatofibrosarcoma in childhood is difficult, and is usually made several years later in early adulthood because of its slow development, lack of symptoms and generally benign appearance. Histological tests and immunohistochemical staining may confirm clinically suspected diagnosis, and in complex cases, cytogenetic studies can help confirm a diagnosis of dermatofibrosarcoma through detection of reciprocal translocation t (17,22), which fuses collagen type Ialpha1 (COLIA1) and platelet-derived growth factor (PDGDFbeta), and which is highly characteristic of dermatofibrosarcoma protuberans. Conventional treatment of dermatofibrosarcoma protuberans consists of extensive surgical excision, but Mohs micrographic surgery is also advocated for removal of certain dermatofibrosarcoma protuberans, while use of tyrosine kinase PDGF receptor inhibitors such as imatinib mesylate (Glivec) is limited to distant metastases.

Adult↗
PubMed2006

A case of primary cutaneous nasal type NK/T-cell lymphoma and review of the literature.

INTRODUCTION: Cutaneous T-cell lymphoma subtypes are now better identified thanks to progress in immunohistochemistry. This article describes a new case of primary cutaneous natural killer/T-cell lymphoma of nasal type (NKTL-NT) and reviews 18 other cases of this rare neoplasm. CASE REPORT: A 79-year-old man presented with a 3-cm nodular tumor of the left leg occurring on a primary chronic lymphedema of the legs. The lesion was CD56+, CD3 intracytoplasmic+, CD45+ and Epstein-Barr virus+. A comprehensive workup including CT scan and bone marrow biopsy was negative and a diagnosis of NKTL-NT with a primary cutaneous involvement was made. The patient was free of disease under multi-agent chemotherapy after 24 months of follow-up. DISCUSSION: After reviewing 18 other cases of primary cutaneous NKTL-NT, we conclude that the prognosis of these lymphomas is usually poor. However, limited cutaneous forms have a longer median survival than extracutaneous variants.

Aged↗
PubMed2005

[Cutaneous CD8+ squamous T-cell bullous lymphoma].

INTRODUCTION: Bullous forms of cutaneous T-cell lymphomas are rare. A new group of cutaneous T-cell lymphomas has recently been identified as a distinct clinicopathological and immunophenotype entity. These cutaneous T-cell lymphomas express a CD8+ phenotype, rarely expressed in other cutaneous T-cell lymphomas. CASE REPORT: We describe a cutaneous CD8+ squamous T-cell lymphoma with polymorphic clinical features, strongly epidermotropic lymphoid infiltrate and spongiosis, classical for this type of lymphoma. DISCUSSION: Bullous lesions in cutaneous T-cell lymphoma should evoke the possibility of a cutaneous CD8+ T-cell lymphoma, once other bullous diseases have been excluded. Spongiosis, rare in other types of T-cell lymphoma, and strongly epidermotropic pleomorphic lymphoid infiltrate are classical histological features. The association of polymorphic lesions, bullas and atypical CD8+ epidermotropic phenotype should evoke this diagnosis even at the early stage. Treatment is difficult and classical chemotherapy often fails. Prognosis is poor with a mean overall survival of 32 months.

Aged↗
PubMed2004

[Agranular CD4+ CD56+ hematodermic neoplasm: a new case report].

BACKGROUND: "Agranular CD4+ CD56+ hematodermic neoplasm" are rare hematologic neoplasms which were recently shown to correspond to the plasmocytoid dendritic cells. CASE REPORT: A 83-year-old presented isolated skin lesions purple, infiltrating the dermis. The biopsy has shown a dense dermal infiltration with malignant cells CD4+ CD56+ CD43+. There were no bone marrow involvement and no circulating blood cells. A chemotherapy permitted a clinical remission after six courses. Unfortunately, skin and blood relapses appear four months later. After a short success of chemotherapy by DHAP, the patient died three month later. DISCUSSION: "Agranular CD4+ CD56+ hematodermic neoplasm" is a distinct entity from the cutaneous primary lymphomas. Recently plasmocytoid monocyte cells have been identified as the precursor of the malignant population with the high expression of CD123, IL3 receptor. It is a distinct clinicopathologic entity by its clinical presentation with skin tropism, bone marrow involvement with or without leukemic phase and poor prognosis independent of the kind of treatment and its particular phenotype CD4+ CD56+ CD43+. It would be interesting to use antibodies linked to CD123 in therapeutic because any treatment have efficacity in this disease.

Aged↗
PubMed2003

Successful treatment with infliximab and methotrexate of pyostomatitis vegetans associated with Crohn's disease.

A 35-year-old woman with severe fistulizing Crohn's disease presented with pyostomatitis vegetans affecting both the mouth and the vulva. The coalescing pustules transformed within several days into vegetating lesions on areas of inflammation. Microbial assessments revealed no pathogenic agent. Histology showed neutrophilic microabscesses, but no granulomas. Three injections of infliximab and maintenance therapy with methotrexate resulted in rapid and complete regression of both the pyostomatitis vegetans and the Crohn's disease. Infliximab and methotrexate may be a promising treatment for the rare cases of pyostomatitis vegetans associated with Crohn's disease.

Adult↗
PubMed2003

Relevance of vertical growth pattern in thin level II cutaneous superficial spreading melanomas.

Thin (<or=0.76 mm) level II cutaneous superficial spreading melanomas (SSMs) are known to be of excellent prognosis and very few recur, metastasize, or are lethal. Although many prognostic features at this stage have been studied, none appears to be statistically significant. The concept of tumor growth phase is correlated with Clark's level except for level II. SSM level II show either an invasive vertical growth phase or an invasive radial growth phase. The aim of our study (retrospective, multicenter, and case-control type) was to investigate the prognostic impact of vertical growth phase in thin level II cutaneous SSM. We identified 12 patients of poor outcome with complete initial excision. Each case was matched with three controls for gender, age, location, tumor thickness, and follow-up period since diagnosis. Independent pathologists studied all cases and controls. Univariate analyses were performed with a conditional logistic regression method. A kappa test was used to assess reproducibility between pathologists. Our study is the first and largest that shows that vertical growth phase is the only statistically significant prognostic factor for thin level II cutaneous SSM. We propose that growth phase evaluation (a minimum of eight serial sections being mandatory not to underdiagnose vertical growth phase) should be added to the recommendations for melanoma histologic report, at least for level II SSM.

Adult↗
PubMed2002

Combined analysis of T cell receptor gamma and immunoglobulin heavy chain gene rearrangements at the single-cell level in lymphomas with dual genotype.

By prospectively studying immunoglobulin heavy chain gene (IgH) and T cell receptor gamma (TCRgamma) gene rearrangements in 398 lymphoma cases, a dual genotype was observed in 13% of B cell and 11% of T cell lymphomas. According to histological subtype, the highest incidence was observed for mantle cell lymphomas (32%) and lymphoplasmacytic lymphoma (21%) among B cell lymphomas, and for angioimmunoblastic lymphoma (AILT) (46%) and Sézary syndrome (SS) (50%) among T cell lymphomas. To determine whether the dual genotype corresponds to the presence of two distinct monoclonal populations or to the presence of both rearrangements within the same lymphoma cells, single-cell microdissection was used after immunohistochemistry and a single-cell combined IgH and TCRgamma gene analysis was designed after a whole-genome amplification step. This protocol was applied to the study of two nodal B cell lymphomas (one diffuse large B cell lymphoma and one mantle cell lymphoma) and two cutaneous T cell lymphomas (one AILT and one SS). Two cases (SS and mantle cell lymphoma) were true bigenotypic lymphomas, as both IgH and TCRgamma monoclonal rearrangements were detected in the same cells. Conversely, in the diffuse large B cell lymphoma and AILT cases, large CD22+ single cells exhibited only the monoclonal IgH rearrangement but not the TCRgamma gene that was detected in CD3+ single cells. Such an approach allows the identification of true bigenotypic lymphoma among dual genotypic lymphoma. Specific genetic alterations may be further amplified from microdissected cryopreserved material, such as the t(11;14) breakpoint detected in bigenotypic B cells of the mantle cell lymphoma case.

Chromosomes, Human, Pair 11↗
PubMed2002

Detection of circulating cells expressing chromogranin A gene transcripts in patients with lung neuroendocrine carcinoma.

High grade lung neuroendocrine carcinomas, like small and large cell neuroendocrine carcinomas, pose therapeutic problems. Most initially respond to chemotherapeutic agents, but early relapses are frequent and are resistant to the presently available treatments. Our study reports for the first time the development and evaluation of a test for detecting the presence of circulating tumour cells by measuring chromogranin A gene transcripts with reverse transcriptase-polymerase chain reaction (RT-PCR) and Southern blotting. The test is specific and sensitive (detection of 10 cancer cells/ml blood), and only minimally invasive. Positivity is statistically correlated to high grade neuroendocrine carcinomas and to a poor prognosis with a 3-fold higher lethal risk. The test now needs to be assessed for its usefulness as a tool in the initial staging procedures and follow-up by comparison with the recent immunoradiometric assay (RIA) for detection of chromogranin A in the serum.

Alternative Splicing↗
PubMed2002

[Bullous localization of a lupus erythematosus induced by radiotherapy].

INTRODUCTION: Connective diseases induced or exacerbated by radiotherapy are not frequent. We report a particular erosive erythematosus lupus with lesions in the precise distribution of radiation therapy given for a breast cancer. OBSERVATION: An 80 year-old woman presented with painful erosive skin lesions of her breast which had been treated by irradiation 9 years before. The patient also had rheumatoid arthritis. A skin biopsy showed keratinocyte necrosis, acantholysis and a dermal lymphocytic infiltrate under the basal cell layer. Direct immunofluorescence showed granular deposition of IgG, IgM and C3 along the basal cell layer. Antinuclear antibodies were positive at a titre of 1: 1,000. Erythematosus lupus diagnosis was established and annular lesions secondarily appeared on her neck, back, and arms. A treatment with hydroxychloroquine and topical corticosteroïds was effective in 3 months. DISCUSSION: The unusual erosive lesions and their localization on a previously irradiated site suggest the role of X-rays in our observation, despite their late appearance after radiotherapy. Radiotherapy and erythematosus lupus affect the same target structures (basal cells and small dermal capillaries) and could have additive effects. The presence of rheumatoid arthritis in the past medical history may have exaggerated this complication.

Adrenal Cortex Hormones↗
PubMed2001

Primary digestive Richter's syndrome.

The clinical and morphologic transformation of 3 to 5% of chronic lymphocytic leukemia (CLL) to diffuse large-cell lymphoma (DLCL) is commonly referred to as Richter's syndrome. Richter's syndrome occurs mostly in lymph nodes and may represent a second neoplasm or a transformation from the same clonal population. Clinical features in six patients with digestive Richter's syndrome were recorded. Paired samples of CLL and DLCL were investigated by immunohistological analysis (n = 6) and by polymerase chain reaction (PCR) for immunoglobulin heavy-chain gene rearrangement (n = 4). Histological examination revealed the involvement of the gastrointestinal tract by DLCL of B-cell phenotype (n = 6). The same monoclonal rearrangement between CLL and DLCL was demonstrated by PCR and sequencing analyses in two patients. The monoclonal rearrangement was different between CLL and DLCL in only one case. Median survival was 22 months for five patients receiving chemotherapy, suggesting that digestive Richter's syndrome has a better prognosis than nodal Richter's syndrome. Indeed, appropriate surgical resection combined with chemotherapy led to partial or complete remission in four patients.

Aged↗
PubMed2001

Evidence that an identical T cell clone in skin and peripheral blood lymphocytes is an independent prognostic factor in primary cutaneous T cell lymphomas.

The monoclonality of the T cell receptor gamma-chain gene was analyzed by polymerase chain reaction in skin and blood specimens of 85 patients with cutaneous T cell lymphomas including 67 mycosis fungoides, seven Sézary syndromes, and 11 CD30- nonepidermotropic cutaneous T cell lymphomas. A cutaneous T cell clone was detected in 69% of mycosis fungoides and 100% of Sézary syndromes. This frequency varied according to the clinical stage: 57% in early stages (Ia-IIa) to 96% in advanced stages (IIb-IV, Sézary syndrome). A peripheral blood T cell clone was detected in 42% of early stages and in 74% of late stages but was identical to the cutaneous one in 15% and in 63%, respectively. A significant association between initial clinical stage and T cell monoclonality was observed. In nonepidermotropic cutaneous T cell lymphomas, T cell monoclonality was detected in 55% of skin and 36% of blood samples. Univariate and multivariate analyses showed that, besides the initial clinical stage, an identical cutaneous and blood T cell clone was an independent prognostic factor for disease progression of mycosis fungoides/Sézary syndrome (hazard ratio 3.4, 95% confidence interval 1.4-9.9). Parallel polymerase chain reaction study of skin and blood specimens may therefore provide an initial prognostic marker that could help to monitor therapeutic strategies. A fully prospective study, with simultaneous therapeutic trials, needs to be done to confirm our findings and to include treatment variables in the statistical analysis.

Adult↗
PubMed2001

Sodium valproate-induced cutaneous pseudolymphoma followed by recurrence with carbamazepine.

We report a patient with a sodium valproate-induced cutaneous pseudolymphoma, presenting with an erythematous papule, histologically mimicking a non-epidermotropic T-cell lymphoma. Polymerase chain reaction study of the skin biopsy revealed monoclonal rearrangement of the T-cell receptor gamma gene. Withdrawal of sodium valproate was followed by regression of the lesion, but 5 months after substitution by carbamazepine, two further papules appeared, with similar histological features and a T-cell clone identical to the initial one. Carbamazepine was stopped and the lesions disappeared without relapse over a 4-year follow-up. Sodium valproate is very rarely responsible for a hypersensitivity syndrome, and our case is the first report of sodium valproate-induced cutaneous pseudolymphoma. The recurrence with carbamazepine may be due to a common effect on T-cell lymphocyte function. The return of the same monoclonal population shows that the recurrence of monoclonal T cells may be observed in benign conditions and is not an exclusive hallmark of cutaneous lymphoma.

Adult↗