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Biomedical subjects

M Beylot-Barry

Publications and source records attributed to M Beylot-Barry.

At least 19 recordsLinked to original sources

[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↗

[Coexistence of dermatomyositis and macrophagic myofasciitis].

INTRODUCTION: An inflammatory myopathy, characterised by joint and muscle pain, chronic asthenia, with infiltration of peri-fascicular epimysium, perimysium and endomysium by cells of the macrophagic line, macrophagic myofasciitis is often associated with other, generally auto-immune, affections. However, the coexistence with another inflammatory myopathy is relatively rare. OBSERVATION: A 29 year-old woman presented with 2 distinct inflammatory myopathies, a macrophagic myofasciitis and a dermatomyositis, which had appeared a few years after. DISCUSSION: In the rare cases in which 2 inflammatory myopathies are combined, the precise relationship between them is unknown, an individual susceptibility to developing muscle diseases is suggested.

Adult↗

[Painful bruising syndrome: a psychogenic disease].

INTRODUCTION: Painful bruising syndrome was described by Gardner and Diamond in 1955. It is marked by spontaneous bruising, without any biological abnormality, affecting young women with pathological mental context. EXEGESIS: We report three observations with painful bruising syndrome. In a patient, psychotherapy induced improvement in dermatological and articular manifestations. In other case, placebotherapy made clinical symptoms go away for a prolonged period. CONCLUSION: Some etiological hypotheses have been postulated for Gardner and Diamond syndrome. However, published cases speak in favour of psychogenic hypothesis. Somatic and psychological approach must be offered to these patients.

Adolescent↗

[Polycythemia vera revealed by Wells syndrome].

BACKGROUND: Wells syndrome is an eosinophilic dermatosis that is mainly reported in association with infections or insect bites, and more rarely during haematological disorders. CASE REPORT: A 32 year-old woman presented several recurrent episodes of massive swelling and erythema papulo-nodular inflammatory plaques on the buttocks that spontaneously resolved. The biopsy revealed marked infiltration of the dermis with eosinophils suggesting Wells syndrome. Examination found an isolated hepatosplenomegaly with true polycythemia and a myeloproliferative disorder. The diagnosis of Vaquez disease was made. DISCUSSION: Wells syndrome is a distinctive disease entity with a wide polymorphism of clinical and histological features, unspecific, and varying depending on the age of the lesions. Various triggering factors are involved, but this syndrome may also reveal hematological disorders. Cutaneous manifestations often occur before the hematological diagnosis, but are frequently misdiagnosed. Only two other cases of Wells syndrome associated with Vaquez disease have been reported. Our case report underlined the importance of systematic research for an hemopathy in Wells syndrome, especially in young patients.

Adult↗

[Strip papular mucinosis associated with systemic sclerosis].

INTRODUCTION: Other than discreet infra-clinical mucinous deposits observed during many inflammatory dermatoses, clinically visible dermal mucinosis can sometimes be associated with collagenosis. It is usually lupus and very rarely scleroderma. In this case, no confusion with papular mucinosis must be made. CASE REPORT: A 51 year-old woman presented with infiltrated erythematous lesions in strips on the inner sides of the thighs and legs, associated with myalgia, arthralgia and puffy fingers. Mixed connective tissue disease was the initial diagnosis. The clinical picture was rapidly completed by sclerodactylia, telangiectasia, a Raynaud's syndrome and esophageal involvement leading to the diagnosis of CREST-type systemic scleroderma. The biopsy of the erythematous strip lesions revealed a dermal mucinosis. Treatment with hydroxychloroquine led to the regression of the mucinous lesions and the stabilization of the scleroderma, which, four years later, had not developed further. DISCUSSION: Dermal mucinosis can accompany lupus erythematosus, in rare cases dermatomyositis and, in exceptional cases, scleroderma. The clinical presentation varies with large infiltrated plaques, reticulated or papulo-nodular lesions. Conversely, strip lesions such as those observed in our patient have never been reported till now. The association of a localized dermal mucinosis and a scleroderma must not lead to the erroneous diagnosis of papular mucinosis of clearly differing prognosis. The occurrence of a mucinosis during collagenosis might be related to enhanced synthesis of mucin by the fibroblasts mediated by the inflammatory cytokines, increased in this context.

Female↗

[Treatment of pemphigus with intravenous immunoglobulin].

INTRODUCTION: The interest of intravenous immunoglobulins (Igi.v.) in the treatment of pemphigus is discussed. Pemphigus is not a recognized indication for this treatment by the CEDIT (French Committee for the assessment and diffusion of technological innovations). The aim of this study was to assess the efficacy of Igi.v. in the treatment of severe corticosteroid-dependent or resistant pemphigus. MATERIAL AND METHODS: A retrospective study using a standardized questionnaire was conducted in the various departments of dermatology among the "Groupe Bulles" of the French Society of Dermatology. The study collected the following information from 12 patients suffering from pemphigus and treated with Igi.v.: 1) general demographical data; 2) characteristics of the pemphigus; 3) different treatments applied, and 4) efficacy and side effects of treatments. RESULTS: Among the 12 patients studied at the end of treatment with Igi.v., 8 were in complete remission (1 fleetingly for 2 months and 1 preceding initiation of Igi.v.), and 2 were improved (1 temporarily for 4 months). A reduction in corticosteroid therapy was possible in 75 p. 100 of cases (9 patients). During treatment with Igi.v., immunosuppressors were combined with oral corticosteroids in 3 cases. It was possible to reduce their dose 1 one case and to stop them in another case. No major side effect related to treatment with Igi.v. was observed. Six months and one year after the treatment, complete remission rates were respectively 6/10 and 5/8. One patient relapsed more than one year after the end of Igi.v. treatment. CONCLUSIONS: Although very expensive, treatment with Igi.v. appears of interest in the treatment of severe corticosteroid-dependent or resistant pemphigus. Moreover tolerance is excellent. The results of our study warrant confirmation in a prospective study.

Adrenal Cortex Hormones↗

[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↗

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↗

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↗

Cutaneous sarcoidosis during interferon alfa and ribavirin treatment of hepatitis C virus infection: two cases.

Interferon-induced sarcoidosis is well documented. We report two new cases of sarcoidosis in two patients with hepatitis C virus infection treated with interferon alfa and ribavirin. These patients developed cutaneous sarcoidosis about 3 months after the beginning of the combination therapy. Spontaneous regression of the lesions was noted after discontinuation of the treatment. There have been more than 20 observations of the appearance or aggravation of this granulomatosis with interferon alfa and more recently with the combination of interferon alfa plus ribavirin. Dermatological signs are found in 50% of cases, and are often diagnostic. Other clinical symptoms of sarcoidosis resemble side-effects of interferon. The evolution is fairly stereotypical and is marked by a regression of the lesions following a dose reduction or curtailment of interferon. Interferon alfa acts by stimulating the T-helper (Th) 1 immune response. In addition to its antiviral action, ribavirin also enhances the Th1 response. Indeed, the superiority of the combination of interferon alfa and ribavirin in terms of antiviral action is corroborated by the enhancement of a Th1-type immune reaction by this combination. At the same time, this immune cell reaction triggers a greater granulomatous reaction.

Adult↗

[Mycobacterial bovis BCG cutaneous infections following mesotherapy: 2 cases].

INTRODUCTION: Infectious complications following mesotherapy are usually due to ordinary bacteria or atypical mycobacteria. We report two new cases of mycobacterial bovis BCG infections following mesotherapy. To our knowledge only one case has already been reported. CASES REPORTS: A 52 year-old woman developed vaccinal MERIEUX BCG cutaneous abscesses following mesotherapy. Identification was made by a novel class of repeated sequences: Mycobacterial interspersed repetitive units. Despite prolonged anti-tuberculous therapy, complete remission was not obtained and surgical excision was performed. The second case was a 49 year-old man who developed a mycobacterial bovis BCG cutaneous abscess (Connaught) after mesotherapy, the regression of which was obtained with anti-tuberculous therapy. DISCUSSION: The severity of these two mycobacterial infections following mesotherapy illustrate the potential risks of mesotherapy. Identification is possible by molecular biology techniques (PCR and sequencing). The origin of this infection is unclear and therapeutic decision is difficult. Some authors recommend anti-tuberculous therapy but surgical excision may be necessary as in our cases.

Abscess↗