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Biomedical subjects

P Souteyrand

Publications and source records attributed to P Souteyrand.

At least 19 recordsLinked to original sources

Epidermolysis bullosa acquisita in a 3 1/2-year-old girl.

A 3 1/2-year-old girl had a subepidermal bullous eruption with immunopathologic features that were consistent with epidermolysis bullosa acquisita or bullous systemic lupus erythematosus. This report highlights the difficulty encountered in distinguishing between epidermolysis bullosa acquisita and other bullous disorders that involve the dermoepidermal junction and the need for modern immunologic investigations in the diagnosis of bullous diseases in children.

Child, Preschool

Efficacy and safety of calcipotriol (MC 903) ointment in psoriasis vulgaris. A randomized, double-blind, right/left comparative, vehicle-controlled study.

BACKGROUND: The biologically active form of vitamin D3, calcitriol, may offer a new therapeutic approach to psoriasis. Calcipotriol, a new vitamin D3 analogue, is at least 100 times less calcemic than calcitriol. OBJECTIVE: Our purpose was to study the efficacy and safety of calcipotriol in the treatment of psoriasis vulgaris. METHODS: In a right/left comparative, double-blind study, treatment with calcipotriol ointment (50 micrograms/gm) twice daily and placebo was given for 4 weeks. The preferred treatment was continued, without opening the code, for another 4 weeks. Efficacy, as measured by the Psoriasis Area and Severity Index and by the investigator's and patient's global assessment, and safety were assessed every 2 weeks. RESULTS: The mean Psoriasis Area and Severity Index fell in 4 weeks from 14.2 to 6.3 with calcipotriol and from 14.1 to 9.2 with placebo (p < 0.001; 95% confidence interval for difference: 1.78-->3.94). Local side effects were equally common with calcipotriol and placebo. The mean serum calcium remained unchanged. CONCLUSION: Topical application of up to 50 gm of calcipotriol ointment per week was found to be an effective and safe treatment of psoriasis vulgaris.

Adult

No evidence for a spirochaetal origin of localized scleroderma.

We looked for evidence of a Borrelia infection in 15 patients with morphoea. We were not able to detect antibodies to Borrelia burgdorferi in any of these 15 patients. None of the 14 skin biopsies examined by immunohistochemistry showed evidence of spirochaetes. Skin biopsies were cultured in 10 patients. All were negative. These results do not support a spirochaetal origin of localized scleroderma.

Adolescent

Hydantoin-induced cutaneous pseudolymphoma with clinical, pathologic, and immunologic aspects of Sézary syndrome.

BACKGROUND: The phenytoin-induced hypersensitivity syndrome is characterized by the development of fever, rash, lymphadenopathy, and hepatitis associated with leukocytosis and eosinophilia. This article describes the unusual occurrence of a pseudo-Sézary syndrome in the days following the introduction of phenytoin treatment. OBSERVATION: A phenytoin-induced erythroderma developed in a 60-year-old woman the histologic, cytologic, and immunologic characteristics of an erythrodermal cutaneous T-cell lymphoma of the Sézary syndrome type with lymph node involvement. The dramatic improvement after withdrawal of drug therapy and the absence of recurrence 5 years after led us to consider it as a hydantoin-induced pseudolymphoma. CONCLUSIONS: Although lymph node pseudolymphomas induced by phenytoin are well known, few cases of hydantoin-induced mycosis fungoides have been reported in the literature. We present herein the first case of a Sézary-like syndrome associated with phenytoin therapy. Such a patient must be monitored regularly because of the risk of a true malignant lymphoma developing even many years later.

Aged

Sequential immunopathologic study of oral lichen planus treated with tretinoin and etretinate.

Twenty-five patients with oral lichen planus confirmed by histologic examination were divided into three groups: 10 patients received topical treatment with tretinoin, 10 received systemic treatment with etretinate, and the five remaining patients, who received no treatment, served as a control group. An immunopathologic study with the use of monoclonal antibodies was carried out before and after the 3-month course of treatment and showed a variation in the distribution and phenotype of inflammatory cells. No difference between the two types of treatment was observed, but treated patients had a shorter evolution of the disease when compared with control patients.

Adolescent

The treatment of 45 patients with cutaneous T-cell lymphoma with low doses of interferon-alpha 2a and etretinate.

Forty-five patients with cutaneous T-cell lymphomas (CTCL), 32 with mycosis fungoides (MF) and 13 with Sézary syndrome (SS), were treated with interferon-alpha 2a (IFN-alpha 2a) (6-9 x 10(6) IU daily) for 3 months. Those responding to treatment were then treated with interferon-alpha alone (6-9 x 10(6) IU three times weekly), and non-responders received a combination of etretinate (0.5 mg/kg/day) and IFN-alpha 2a in similar concentrations. After 12 months of treatment, 28/45 patients (62.2%) were in complete or partial (greater than 50%) remission. Of these, 17 (60.7%) were receiving IFN-alpha alone and 11 the combined interferon-retinoid therapy. Of the patients with MF stage I and II, 20/25 were responders (12 receiving IFN-alpha alone and eight on combined therapy), whereas only 8/20 with stage IV or SS responded to treatment (five receiving IFN-alpha 2a alone and three combined therapy). These results suggest that the association of etretinate with low-dose recombinant IFN-alpha 2a is an effective means of treating epidermotropic CTCL, particularly in the early stages.

Adolescent

[Aging and the cutaneous immune system].

The changes in the immune system during the ageing process have been described in numerous publications, which are sometimes contradictory. The study of cell mediated immunity and of hormonal immunity provide only a partial reflection of the smooth working of the cutaneous immune system, which is dependent on fragile cell interactions between T lymphocytes, cells of Langerhans and keratinocytes, which involves numerous soluble mediators. Three possible mechanisms leading to or contributing to the decline in immune function with age should be considered: a modification of the distribution of lymphocyte subpopulations (the T helper lymphocytes and T-suppressing lymphocytes are affected by age in particular), changes in the mechanisms of suppression and changes in the maturation of B and T lymphocytes.

Antibody Formation

Clonotypic heterogeneity in cutaneous T-cell lymphomas.

The antigen receptor genes studied (immunoglobulin gene for B-cells, and T-cell receptor -beta or -gamma gene for T-cells) represent the most powerful tools for diagnosing the clonality of a lymphoid lineage. We have clonotyped 23 cutaneous T-cell lymphomas and 5 were found to be clonotypically all heterogeneous. Analysis of each patient was performed either from serial skin biopsies taken several months apart or from different tumor samples. In these cases, T-cell lymphoma clonotypic heterogeneity was demonstrated and was especially evident when examining different tumor sites. Moreover, in one case, a biogenotypic population (immunoglobulin and T-cell receptor-rearranged) was found. This unexpected high frequency of T-cell clonal heterogeneity (22%) could be explained either by the evolution of subclones from a single undifferentiated malignant cell or by the independent transformation to cancer of 2 or more lymphocytes, though the latter seems less likely. Clonotypic heterogeneity seems to be as frequent in T-cell lymphomas with cutaneous lesions as in B-cell leukemias.

Gene Rearrangement

Dual genotype in cutaneous T-cell lymphomas and pseudolymphomas.

Genomic DNA digests of skin biopsies from 20 patients with cutaneous T-cell lymphomas and pseudolymphomas were studied by hybridization, using probes for the constant region of the T-cell receptor beta chain and the joining region of the immunoglobin heavy chain gene. Skin biopsies from all 20 patients contained a monoclonal T-cell population. In addition, DNA from 5 patients contained an immunoglobulin gene rearrangement. These results demonstrate that cutaneous T-cell lymphomas are clonal T-cell malignancies that frequently express a dual genotype, which may sometimes reflect the clonotypic heterogeneity of these disorders.

Gene Rearrangement, beta-Chain T-Cell Antigen Rece