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Biomedical subjects

E Thomine

Publications and source records attributed to E Thomine.

At least 19 recordsLinked to original sources
PubMed1995

Primary cutaneous medium and large cell lymphomas other than mycosis fungoides. An immunohistological and follow-up study on 54 cases. French Study Group for Cutaneous Lymphomas.

Primary cutaneous medium and large cell lymphomas (MLCL) other than mycosis fungoides (MF) are rare, and their prognosis and treatment are controversial. The clinical, immunohistological and follow-up data of 54 well-documented cases of primary cutaneous MLCL other than MF, seen in our institutions over a 14-year period, were retrospectively reviewed, in order to determine the prognostic factors related to these lymphomas, and to analyse the results obtained with different treatment regimens. Forty-six patients presented with a solitary tumour or with localized lesions, and eight had disseminated cutaneous lesions. According to the updated Kiel classification, 45 cases (83%) corresponded to B-cell lymphomas: centroblastic lymphomas, 32 cases; centroblastic-centrocytic lymphomas, 11 cases; immunoblastic lymphomas, two cases. Nine cases (17%) were classified as T-cell lymphomas: pleomorphic medium and large cell lymphomas, eight cases; anaplastic large cell lymphoma, one case. Four of eight patients with disseminated skin lesions had a T-cell lymphoma, whereas 41 of 46 patients with a solitary tumour had a B-cell lymphoma. Patients with disseminated skin lesions and elevated serum lactate dehydrogenase (LDH) levels had a poor prognosis. Comparison of patients' overall survival, depending on immunohistological subtype, showed that the median survival of patients with pleomorphic T-cell lymphoma was 2.5 years, whereas it was not reached at 12 years for patients with centroblastic-centrocytic and centroblastic lymphoma. The eight patients with disseminated skin lesions were treated with polychemotherapy.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1995

Catastrophic antiphospholipid syndrome with fatal acute course in rheumatoid arthritis.

A 34-year old woman, with a 3 yr history of severe seropositive rheumatoid arthritis (RA) with lupus anticoagulant and anticardiolipin antibodies, developed a massive anterior myocardial infarction and ischemia of the lower extremities, with disseminated intravascular coagulation resulting from extensive tissue damage. Seven days after admission, she died of severe heart failure complicated by ventricular fibrillation. To our knowledge, this is the first documented case of fatal acute antiphospholipid syndrome in RA.

Adult↗
PubMed1995

[Association of lichen sclerosus and monomelic scleroderma].

INTRODUCTION: Coexistence of lichen sclerosus and scleroderma is well demonstrated. However, clinical and histological lesions of lichen sclerosus and scleroderma, in a linear pattern, do not seem to have been reported. CASE REPORT: We report the case in a six years old boy, of both lichen sclerosus and linear scleroderma lesion that involved his left lower limb. Immunological and inflammatory investigation was normal or negative, as well as, bone and muscles analyze of the leg. Further outcome was benign. DISCUSSION: This new case confirms the possibility of an association, inside a same limb, of lichen sclerosus and localized scleroderma, and supports the concept of a common etiological process in these two diseases. In an other hand, it could reflect the possibility of two distinct clinical expressions of the same pathogenic process.

Atrophy↗
PubMed1995

[Childhood acromelalgia a propos of a case revealing Fabry's disease].

The occurrence of an acrosyndrome (Raynaud's phenomenon, erythermalgia, acrodynia...) in childhood may be the first manifestation of a general disease. Though it can be an early onset Raynaud's disease, it could also be the first sign of a connective tissue disease (juvenile polyarthritis, mixed connectivitis...) or of a overload disorder. We report a case of childhood-onset acromelalgia leading to the discovery of Fabry's disease. This chromosome X-linked hereditary disorder, resulting in the ubiquitous accumulation of neutral sphingolipids, is usually rapidly suspected by the finding of "boxer-short" angiokeratoma. Diagnosis is confirmed by the ophthalmic examination (cornea verticillata), by the pathological examination of a skin sample, and by the measure of alpha-galactosidase A activity. Treatment is usually only symptomatic, but the discovery of the mutations responsible for the disease could open the way to specific therapy.

Adult↗
PubMed1995

[Pemphigoid mimicking epidermolysis bullosa acquisita].

INTRODUCTION: Subepidermal autoimmune bullous dermatoses form a clinical entity for which there is not always an individualized clinical and pathological description. CASE REPORT: A patient presented with bullous skin disease of atypical nature. There was an almost total desepidermization of the legs, vast areas of erosion on the trunk and arms with a Nikolski sign in an area of healthy skin, buccal involvement, multiple milium cysts and ungueal dystrophies with nail loss. DISCUSSION: This clinical presentation in this patient suggested acquired bullous epidermolysis. However, according to the recently defined clinical criteria for pemphigoid, the probability of correct diagnosis of pemphigoid was greater than 95 p. 100 since nearly three fourths of the major criteria were present. This diagnosis was confirmed by reference techniques (electron microscopy, indirect electron immunomicroscopy and immunoblotting). Thus, bullous autoimmune diseases of the dermoepidermal junction can be reliably differentiated on the bases of the clinical features, together with direct and indirect immunofluorescence on salt-split skin.

Aged↗
PubMed1992

Production of a human monoclonal anti-epithelial cell surface antibody derived from a patient with pemphigus vulgaris.

The production of monoclonal autoantibodies derived from individuals with autoimmune diseases constitutes a powerful tool to analyse an autoimmune process at both the antigen and antibody levels. We established a human anti-epithelial cell surface monoclonal antibody by applying hybridoma technology using peripheral blood lymphocytes from a patient with pemphigus vulgaris using a heteromyeloma as the fusion partner. The F12 monoclonal antibody displays four major characteristics: (1) it belongs to the IgM, kappa class; (2) it binds to the cell surface of stratified squamous and simple epithelia; (3) it recognizes an antigenic determinant associated with the desmosomal complex as demonstrated by indirect immunoelectron microscopy; (4) by immunoblotting analysis, it reacts with a 185 kDa polypeptide which was also recognized by a few pemphigus vulgaris sera. Although the F12 monoclonal antibody does not have the immunochemical properties of classical pemphigus vulgaris autoantibodies, several arguments suggest its relevance to the pemphigus vulgaris autoimmune response and, therefore, the heterogeneity of the antigen/antibody systems involved in this autoimmune disorder.

Adolescent↗
PubMed1992

[Benign glandular schwannoma and Recklinghausen disease. Report of a case].

The authors describe the case of a 32 year-old woman with a congenital neurofibromatosis referred for excision of a painful subcutaneous nodule of the left popliteal space. The histological study showed a tumor displaying a biphasic pattern made of fascicles of benign spindle cells admixed with mucinous epithelium. Immunohistochemistry (cytokeratin X, ECA X) and electron microscopy helped to prove the true epithelial nature of the glandular components. Glandular schwannoma has rarely been described, especially in a benign histological pattern; this one has never been reported in association with Von Recklinghausen's disease. The histogenesis of the glandular schwannoma remains uncertain.

Adult↗
PubMed1991

[Osteoarticular manifestations of palmoplantaris pustulosis. A prospective study of 15 cases].

Between 1986 and 1989, we conducted a clinical, biochemical, radiological and scintigraphic prospective study of 15 patients (8 men, 7 women) with histologically proven palmoplantar pustulosis. In 70 percent of the cases the time interval between the first cutaneous and the first osteoarticular signs was 2 years. Anterior thoracic clinical manifestations were frequent. The joints and the numbers of patients involved were: sternoclavicular (12), manubriosternal (6), sternocostal (5), intervertebral (11), sacroiliac (6) and peripheral (10). Two patients had osteitis. The clinical, radiological and scintigraphic findings, as well as the distribution of these arthropathies over the anterior thorax (i.e. over a sternocostoclavicular complex with numerous ligaments), suggest a preference for entheses. Despite the absence of link with the HLA B27 antigen, the frequent association with pelvic and spinal lesions indicate that the articular disease of palmoplantar pustulosis is a spondyloarthropathy.

Adolescent↗
PubMed1991

[Spontaneous rupture of subclavian artery disclosing Ehlers-Danlos disease. A case].

The authors report the case of a 30-year old man who suffered spontaneous rupture of the right subclavian artery. Treatment consisted of carotid-axillary graft since the fragility of the vessel precluded direct suture. The clinical symptoms, together with histological and ultrastructural examinations led to a diagnosis of Ehlers-Danlos syndrome with purely arterial manifestations.

Adult↗