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Biomedical subjects

M Beylot-Barry

Publications and source records attributed to M Beylot-Barry.

89 records · Page 5Linked to original sources

[Sweet syndrome associated with Crohn disease].

The association of Sweet's syndrome and Crohn's disease is rare. We report a new case of such association. A 45 year-old woman developed a diarrhea, fever, and skin lesions consistent with a presumptive diagnosis of Sweet's syndrome. Crohn's disease was also diagnosed. Oral prednisone, associated with mesalazine, effected improvement of both cutaneous lesions and bowel disease. The ten cases of the literature and ours show that Sweet's syndrome may occur during an acute phase of Crohn's disease. Most of the time, Crohn's disease has already been diagnosed. However, this was not so with our patient, wherein lies the originality of our case. A general corticotherapy is the preferred course of treatment.

Crohn Disease↗

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↗

[Dyshidrotic pemphigoid].

INTRODUCTION: Clinical expression of pemphigoid can sometimes be misleading. Localized dyshidrosis is rarely reported in the literature. CASE REPORT: We observed 4 cases of pemphigoid with unusual dyshidrosis lesions. Skin biopsy was unspecific in both cases. Immunofluorescence gave the diagnosis in both. One case responded to low-dose general corticosteroids associated with local applications of corticosteroids. The other patients required high-dose oral corticosteroids. CONCLUSION: Our cases illustrated the misleading dyshidrosis nature of certain pemphigoids and the need for repeated skin biopsies with immunofluorescence in case of recent development of dyshidrosis in elderly subjects. As in the literature, and despite the localized nature of the lesions, general corticosteroid treatment appears necessary in many cases.

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↗

[Lupus bronchiolitis obliterans: diagnostic difficulties].

Bronchiolitis obliterans is exceptional in systemic lupus erythematosus (SLE). Its response to treatment is irregular and prognosis is bad. We report a case of a bronchiolitis obliterans in a 18 year-old woman with a SLE. Oral prednisone, plasmapheresis and cyclophosphamide were unsuccessful. However, only intravenous bolus of methylprednisolone effected improvement.

Adolescent↗

[Arterial involvements in hereditary dysplasia of the connective tissue].

Arterial involvement is an important feature of the diagnosis and, above all, prognosis of heritable disorders of connective tissue. In pseudoxanthoma elasticum, a progressive occlusive syndrome is associated with hemorrhage and especially with gastrointestinal bleeding. Aneurysms are uncommon. Hypertension occurs frequently. Cutaneous signs (yellowish pseudo xanthomatous papules of the large folds) the ocular changes (angioid streaks) and pathology showing numerous, thickened, fragmented, disorganized, calcified elastic fibers in the deep dermis and arterial walls, allow the diagnosis to be made. In the heterogeneous group of Ehlers-Danlos syndromes, type IV is characterized by sudden spontaneous rupture of the large arteries. Aneurysms and carotido-cavernous fistulae are rather frequent. Owing to friability of the arterial walls, arteriograms and other procedure requiring arterial puncture may prove hazardous and surgery difficult. Such patients have an acrogeric morphotype, and thin, fragile skin, but cutaneous hyperelasticity and joint hyperlaxity are usually minimal. Pathology evidences collagen hypoplasia in the skin and arterial walls. The severity of Marfan syndrome is due to aortic involvement. A fusiform aneurysm of the ascending aorta represents a vital risk of rupture. Aortic root dilatation is associated and responsible of severe aortic regurgitation. Aortic dissection is also a serious threat. Improved surgical techniques for repairing a dilated or dissected aortic root with simultaneous replacement of the aortic valve increases the life expectancy of such patients. Dolichomorphism is the characteristic skeletal abnormality, particularly with arachnodactyly and upward ectopia lentis, which is almost bilateral, is a very frequent feature of Marfan syndrome. The most typical histological finding is aortic cystic median necrosis. The basic defect in Marfan syndrome concerns the fibrillin, whose gene is located on chromosome 15. The three diseases detailed in this paper constitute the main areas of this subject, but arterial involvement may occur in other inheritable disorders of connective tissue (osteogenesis imperfecta, cutis laxa, Werner syndrome, Menkes syndrome, etc).

Arteries↗

Human immunodeficiency virus type 1 replication within cystic lymphoepithelial lesion of the salivary gland.

Cystic lymphoepithelial lesions of salivary glands (CLLSG) are nodular or diffuse salivary gland enlargements that are observed in patients who tested positive for human immunodeficiency virus type 1 (HIV-1). Two cases of CLLSG are reported. Particular emphasis is placed on the presence of HIV-1 major-core protein (P24), HIV-1 RNA sequences, Epstein-Barr virus (EBV) DNA sequences, and lymphocyte receptor gene rearrangement. Lymphoid alterations consisted of explosive hyperplasia with a prominent follicular reticular dendritic cell (DRC) network and numerous intrafollicular CD8+ lymphocytes. Intrafollicular DRC strongly expressed HIV-1 major-core protein and HIV-1 RNA, indicating that most DRCs actively replicated the HIV-1 virus. The presence of active HIV-1 replication within DRC and the absence of clonal EBV infected lymphoid population strongly suggest that CLLSG pathogenesis is primarily induced by HIV-1. The presence of oligoclonal immunoglobulin gene rearrangements in our cases, however, suggest the need of long-term follow-up of such patients to determine whether CLLSG could be a benign prelymphomatous disease.

Adult↗

Inflammatory familial palmoplantar keratoderma: Greither's disease?

A case of a familial palmoplantar inflammatory keratoderma with autosomal dominant inheritance is reported. Associated clinical features included vasomotor troubles and hyperhidrosis consistent with a diagnosis of Greither's disease. Light microscopy was nonspecific. Electron microscopy showed aggregated tonofilaments around the nucleus, without true clumps. Desmosomes were numerous and cell-cell junctions showed an imbricated pattern, well demonstrated in the stratum corneum. The diagnosis of Greither's keratoderma is discussed.

Adult↗

[Cutaneous T-cell lymphoma bullosa: 2 cases].

INTRODUCTION: Since the first case described by Kaposi in 1887, bullous forms of cutaneous T-cell lymphomas are extremely. CASE-REPORT: We describe an unusual case of mycosis fungoides bullosa with palmoplantar dyshidrosis-like eruption and, to our knowledge, the first case of pleomorphic cutaneous T-cell lymphoma bullosa. DISCUSSION: Cutaneous T-cell lymphoma bullosa can be very misleading, particularly when the bullous lesions are inaugural. Differential diagnosis have to be excluded (autoimmune blistering diseases or contact dermatitis during topical treatment of lymphoma or bacterial or viral cutaneous infections) by biopsy, direct immunofluorescence and bacteriological examinations. In these cutaneous lymphoma bullosa, the blisters appear to de due to excessive epidermotropism and/or toxicity of the tumoral infiltrate.

Aged↗