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Biomedical subjects

B Vergier

Publications and source records attributed to B Vergier.

At least 55 records · Page 3Linked to original sources

[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↗

CD30-positive cutaneous large cell lymphomas. A comparative study of clinicopathologic and molecular features of 16 cases.

The authors have analyzed and compared the clinicopathologic and molecular features of 16 cases of large cell cutaneous lymphomas expressing CD30 antigen. Three main clinical groups were defined: (1) a group of localized skin disease (7 cases); (2) a group of multicentric skin disease (5 cases); and (3) a group of concomitant skin and extracutaneous disease. Good prognosis was associated with localized skin disease and no history of lymphoma. Interestingly, a majority of Reed Sternberg-like cells was only observed in this group (5 of 6 cases). The two other groups did not show distinctive evolutive nor morphologic features. Southern blot and/or polymerase chain reaction (PCR) technique showed clonality and a T-cell genotype in respectively 13 of 14 and 12 of 12 analyzed cases. Viral infection of tumoral cells was investigated by PCR, in situ hybridization (ISH) or electron microscopy. Epstein-Barr virus (EBV) sequences were detected by PCR and ISH in tumoral cells of cutaneous lesions in one case of skin lymphoma with extracutaneous spreading. No EBV sequence was detected by ISH in the localized lymphomas, whereas HIV particles were visible in tumoral cells in one of these cases. No human T-cell lymphotropic virus (HTLV) tax sequence was amplified by PCR in any case of our series. Our results confirm that CD30-positive cutaneous large cell lymphomas are different clinical and molecular entities. However, a combined clinical and morphologic analysis may help to identify a subset of CD30 cutaneous lymphomas with favorable prognosis.

Adolescent↗

Histiocytic sarcoma that mimics benign histiocytosis.

A 28-year-old man presented with a histiocytic sarcoma of a very uncommon origin, as it had developed for several years like a benign cutaneous histiocytosis resembling generalized eruptive histiocytoma before becoming acute, with nodal and massive pulmonary involvement. Despite various chemotherapies, the patient died within 8 months. Skin biopsies showed histiocytic proliferation in the dermis and node biopsies showed histiocytic proliferation with a sinusoidal pattern. Immunohistochemical analysis, performed on paraffin-embedded sections, demonstrated strong labeling of tumoral cells for CD68 and moderate labeling for CD3 and CD4. CD30 labeling was negative. S-100 protein was positive on a Langerhans' cell reactive subpopulation. Electron microscopy confirmed the histiocytic nature of malignant cells and showed cytoplasmic inclusions such as regularly laminated bodies, dense bodies and pleomorphic inclusions. No Birbeck granules were seen. A gene rearrangement study of T-cell receptor gamma and immunoglobulin heavy chain genes showed a germline configuration. Histiocytic sarcoma is an extremely rare true histiocytic malignancy, the existence of which has been recently debated since it has often been mistaken in the past for large cell lymphomas. Such a deceptive onset as benign cutaneous histiocytosis has not been described in the literature to our knowledge.

Adult↗

Gluteal pigmentation after intramuscular iron injections.

We report a case of gluteal pigmentation after intramuscular iron injections. A study in light microscopy, electron microscopy and X-ray micro-analysis by energy selection confirmed iron deposition. These cutaneous pigmentations with iron deposits are infrequent because such injections are now rarely used.

Adult↗

Pilotropic cutaneous T-cell lymphoma without mucinosis. A variant of mycosis fungoides? French Study Group of Cutaneous Lymphomas.

BACKGROUND: In the course of mycosis fungoides, pilofollicular manifestations without mucinosis (papules, keratoses, comedones, or epidermal cysts) are rare (15 cases reported). Therefore, histological and clinical diagnoses may be difficult. The clinical course and histopathological and immunohistochemical findings in 9 patients are described. OBSERVATIONS: Pilofollicular lesions were present at the onset (n = 3), before (n = 3), or during a relapse of mycosis fungoides (n = 3). Comedones and epidermal cysts were most frequent (n = 5). They disappeared with lymphoma therapy (n = 4), therapy with isotretinoin (n = 3), or spontaneously (n = 1), or they persisted (n = 1). Clues to the histopathological diagnosis consisted of pilotropism of the infiltrate with minor alteration of the hair follicle walls. The infiltrate was monomorphous and composed of sezariform CD4+ lymphocytes. Pilotropic or peripilofollicular infiltrates, or the absence of infiltrate, were detected in consecutive biopsy specimens obtained from the same patient. The keratinocyte expression of intercellular adhesion molecule type 1 was observed in the hair follicle bulb in front of the pilotropic infiltrate but not in the epidermis. No staining was observed in biopsy specimens of 6 of 7 patients with follicular mucinosis, of folliculitis lesions, or of normal hair follicles. CONCLUSIONS: Our findings indicate the role of adhesion molecules in pilotropism leading to mechanical obstruction of the follicle by tumoral cells followed by hyperkeratosis and cyst formation. It remains to be determined if the expression of intercellular adhesion molecule type 1 is the cause or the consequence of pilotropism. By becoming more aware of it, this variant of mycosis fungoides is probably not so rare.

Adult↗

Detection of t(2;5)(p23;q35) translocation by reverse transcriptase polymerase chain reaction and in situ hybridization in CD30-positive primary cutaneous lymphoma and lymphomatoid papulosis.

The t(2;5) generates a chimeric NPM-ALK transcript encoded by the nucleophosmin NPM gene fused to the anaplastic lymphoma kinase gene ALK. Using a reverse transcriptase nested polymerase chain reaction assay we have detected NPM-ALK transcripts within CD30+ primary cutaneous lymphoma and lymphomatoid papulosis (LP). The t(2;5) was identified in 4 out of 9 CD30+ anaplastic lymphomas and in 1 out of 4 CD30+ pleomorphic lymphomas. Moreover, the t(2;5) was detected in 3 out of 10 LPs. All NPM-ALK-positive lymphomas and 1 NPM-ALK-positive LP exhibited a clonal rearrangement of the T cell receptor gamma-chain gene. The t(2;5) was detected in 2 cases of LP without other evidence for a clonal lymphoid population. To identify cells carrying the t(2;5) translocation, we used immunohistochemistry to detect the ALK-encoded p80 protein and in situ hybridization for the specific detection of NPM-ALK transcripts. Both p80 protein and NPM-ALK transcripts were expressed by anaplastic or large CD30+ lymphoma cells with positive NPM-ALK amplification. The presence of t(2;5) in a subset of CD30+ cutaneous lymphoma and LP may indicate a common pathogenesis with a subset of anaplastic nodal lymphoma.

Adult↗

Contribution of histopathologic and molecular analyses to the diagnosis of cutaneous B-cell infiltrates.

To evaluate the value of morphologic, immunohistochemical and molecular analyses, we studied 21 skin biopsy specimens from 19 patients with primary cutaneous B-cell infiltrates. Morphologic review by two independent dermatopathologists confirmed the consensus diagnoses of lymphoma (n = 6) or benign lymphoid hyperplasia (n = 6). A discordant diagnosis was made for the other samples (n = 9), which were thereafter considered as unclassified lymphoid infiltrates. Immunohistochemical analysis showed either a monotypic expression of immunoglobulin light chain or a positive staining with anti-bcl-2 antibodies in three and four samples, respectively, of lymphoma. Polymerase chain reaction was used to analyze immunoglobulin heavy chain and T-cell receptor gamma chain gene rearrangement and to amplify t(14;18) and t(11;14) break points. A clonal molecular marker was detected in 12 of 19 patients. Among these 12 patients, a final diagnosis of lymphoma was confirmed in 8 patients, including the 6 with a morphologic diagnosis of lymphoma. Two patients with clonal benign lymphoid hyperplasia and two with clonal unclassified lymphoid infiltrate presented a benign clinical outcome; one patient was lost to follow-up. Alternatively, no clonal molecular marker was found in two of the patients with lymphoma. The morphologic and molecular criteria, therefore, provided complementary and partially overlapping information for the diagnosis of cutaneous B-cell infiltrates. We proposed a practical use for these data.

Adolescent↗

Ki-1-positive large-cell cutaneous lymphoma mimicking a Stewart-Treves angiosarcoma.

Anaplastic large-cell cutaneous lymphomas (ALCL) represent a heterogeneous group. Primitive cutaneous lymphomas are exceptional, sometimes with a deceptive clinical aspect, but must be considered as a distinct clinicopathologic entity. We report a case of a cutaneous ALCL, appearing 7 years after a homolateral Hodgkin ganglionic lymphoma and occurring on a chronic lymphoedema of the arm. A Stewart-Treves angiosarcoma was suggested owing to its location, vascular aspect and low histologic differentiation. Immunohistochemistry was helpful in making the diagnosis of ALCL. Southern blotting analysis revealed a rearrangement of the beta-chain of the T-receptor gene, thus suggesting the T-cell origin of this lymphoma.

Aged↗

[Necrotic purpura of the head disclosing Churg-Strauss syndrome].

INTRODUCTION: Among the cutaneous manifestations of the Churg-Strauss syndrome, palpable purpura of the extremities is the most common. Although they are not specific, sub-cutaneous nodules of the scalp are the most distinctive lesions. CASE REPORT: A 58 year-old man, with a 7-years history of asthma, developed profuse cutaneous lesions involving the face and the scalp, initially erythematosus and vesiculous, then purpuric and necrotic which led to the diagnostic of Churg-Strauss syndrome. COMMENTARY: Cutaneous lesions occur frequently during the Churg-Strauss syndrome and may reveal the disease as in our patient. However, the initial clinical presentation was uncommon with erythematosus and vesiculous lesions mimicking primary herpes infection. Cutaneous biopsy was useful leading to an early diagnosis.

Churg-Strauss Syndrome↗

p53 oncoprotein expression in cutaneous lymphoproliferations.

BACKGROUND AND DESIGN: p53 overexpression has been reported in systemic lymphoproliferations, but our study is the first large series, to our knowledge, dealing with p53 expression in primary cutaneous lymphomas. p53 immunohistochemical detection using DO7 monoclonal antibody was performed in 54 cutaneous lymphoproliferative disorders, including 37 primary cutaneous T- or B-cell lymphomas. RESULTS: No expression of p53 was found in 14 mycosis fungoides and seven pleomorphic T-cell lymphomas of either low or high grade of malignancy. Furthermore, no p53-positive cells were shown in low-grade B-cell lymphomas and in benign chronic dermatoses. p53 overexpression was seen over lymphomatous cells in 10 cutaneous lymphomas of high grade of T- or B-cell origin with two immunoblastic, three centroblastic, and five anaplastic lymphomas. Five of them occurred in human immunodeficiency virus-infected individuals, suggesting a possible interaction between p53 and viral proteins. p53 immunoreactivity was found over Reed-Sternberg-like cells in two cases of lymphomatoid papulosis. A labeling of keratinocytes located mainly in basal cell layers of the epidermis was observed in only five cases regardless of the lymphoma histologic subtype. CONCLUSIONS: p53 overexpression was not found in the most frequent primary cutaneous lymphomas, epidermotropic T-cell lymphomas. Alternatively, p53 immunoreactivity was observed in most primary cutaneous lymphomas of high grade of malignancy occurring in individuals either infected or not infected by human immunodeficiency virus. Molecular studies will determine if p53 overexpression is associated with p53 gene alteration and help to understand the role of p53 in primary cutaneous lymphoma genesis.

Humans↗

[Lymphoma with skin manifestations in HIV infection: 8 cases].

INTRODUCTION: Cutaneous lymphomas occurring in HIV infection are a rare disease. Most of them are high grade lymphomas with fulminant course and poor prognosis. OBJECTIVE: Evaluate clinical and histological aspects as well as immunophenotype and evolution of these lymphomas. PATIENTS AND METHODS: Eight patients with HIV infection were studied between 1992 and 1994. The clinical and histological features were reviewed by the members of the French Study Group for Cutaneous Lymphomas. Staging procedures for lymphomas were performed in 7/8 patients. RESULTS: Seven non epidermotropic lymphomas and one mycosis fungoides were reviewed. Patients were male (6 cases) and female (2 cases); their mean age was 45 years (27-63). The mean level of CD4 T cells/mm3 was 141 (20-380). Only one patient presented with extracutaneous lesions. These lesions were similar to seronegative patients, but unusual features were observed in two cases. Histological classification showed high grade lymphomas in 6/8 cases. The immunophenotype was: T-cell lymphoma in 4 cases, B-cell lymphoma in 3 cases; it could not be determinated in one case. Six patients died. The median of survival is 8 months in this series. DISCUSSION: Our series confirms the predominance of high grade lymphomas presenting in the skin. The T-cell phenotype is more frequent. The onset of a cutaneous lymphoma has a poor prognosis in HIV infection. Most of our patients had localised disease at presentation. Therapeutic management of these lymphomas must be codified.

Adult↗

[Lichen planus and vaccination against hepatitis B].

INTRODUCTION: The association of lichen planus with liver diseases is now well established. Lichen planus following hepatitis B vaccination are much more unusual. We report here the fifth case of this kind. CASE REPORT: A 16 years old girl developed a purely cutaneous lichen planus one week after the first injection of hepatitis B vaccine Gen Hevac B (Institut Pasteur), which appeared again 3 days after the second injection. The histologic features shown lichenoid pattern with intense keratinocytes necrosis more in favor of lichenoid drug eruption than lichen planus. DISCUSSION: According to our knowledge, only four similar cases have been previously reported. Comparison between the different vaccines used shows that only the HBs antigen and its epitope S could be involved in the lichen planus eruption. Our case is specific due to the early appearance of the eruption after the first injection and by its histologic features. CONCLUSION: New cases of lichen planus following hepatitis B vaccination should help to explain the causal relationship between lichen planus eruption and hepatitis B vaccination.

Adolescent↗

[Study of B-lymphocyte clonality using in vitro gene amplification (PCR) in paraffin embedded samples].

Family-specific primers were used in polymerase chain reaction (PCR) to analyse clonality of immunoglobulin heavy chain (IgH) gene rearrangement. DNA templates were extracted from formalin-fixed paraffin-embedded biopsies from B-cell lymphomas (n = 19), T-cell lymphomas (n = 3), reactive lymphadenopathies (n = 20) and negative controls (n = 2). PCR was also performed on DNA extracted from fragments of the same biopsies that were either fixed in Bouin's solution (n = 24) or snap-frozen (n = 22). The latter were also studied for IgH gene rearrangement by the Southern blot technique. Polyclonal or clonal fragments were obtained from all frozen biopsies. No amplification was observed in 3 out of 44 formalin fixed specimens and 18 out of 24 Bouin fixed specimens. A clonal amplification was only observed in 16 out of 19 formalin-fixed B-cell lymphoma specimens. Therefore this technique allows to study B-cell clonality from formalin-fixed, embedded material.

Acetates↗