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Biomedical subjects

B Flageul

Publications and source records attributed to B Flageul.

At least 37 records · Page 2Linked to original sources
PubMed1998

Treatment of recalcitrant plaque psoriasis with a humanized non-depleting antibody to CD4.

The presence of activated CD4(+) T lymphocytes in psoriatic skin plaques suggests an immune-mediated pathogenesis for the disease. Six patients with recalcitrant plaque psoriasis (PASI>12) received a humanized non-depleting monoclonal antibody to CD4 (ORTHOCLONE OKT(R)cdr4a). The antibody was well tolerated. Four weeks from treatment, the mean decrease in PASI score was 46%. In three patients disease remission was prolonged for up to 6 months and, in one case, up to 1 year post-treatment. In all patients, circulating CD4+ T-cell counts remained normal and peripheral OKTcdr4a-coated CD4+ lymphocytes were detected up to 10 days after antibody infusion. These results point to the relevance of CD4+ lymphocytes in psoriasis. They also emphasize that depletion of CD4+ cells is not mandatory to achieve therapeutic effectiveness.

Adult↗
PubMed1998

Lichen planus pemphigoides is a heterogeneous disease: a report of five cases studied by immunoelectron microscopy.

Lichen planus pemphigoides (LPP) is a rare and controversial disease. It is characterized by bullae arising on lichen planus papules and on uninvolved skin, subepidermal bullae in histology, and linear deposits of IgG and C3 along the basal membrane zone on immunofluorescence of peribullous skin. Our goal was to identify the localization of the target antigen in cases of LPP. Five patients diagnosed with LPP on clinical, histological and immunofluorescence criteria were explored by immunoelectron microscopy and immunoblot. Our results show that the target antigen in LPP is not unique. The localization of the immune deposits was consistent with a diagnosis of bullous pemphigoid in two cases, of cicatricial pemphigoid in two cases and of epidermolysis bullosa acquisita in one case. Our study supports the view that LPP is a heterogeneous condition in which lichen planus may induce different subepidermal acquired bullous dermatoses.

Adolescent↗
PubMed1998

[Adult T-cell lymphoma associated with HTLV-1: a familial form].

BACKGROUND: Adult T-cell leukemia-lymphoma (ATL) can occur in siblings infected with HTLV-1. CASE REPORTS: Two Caribbean siblings developed ATL a few years apart. One case has been reported previously. Both individuals had peripheral lymph node T-cell lymphoma and a few atypical lymphocytes on blood smear. Lymphocytosis, bone marrow biopsy, abdominal computed tomographic scanning, and chest radiography were normal. Clonal rearrangement of T-cell receptor was present in skin lesions for both patients and in the blood for one. HTLV-1 serology was positive. Clonal integration of HTLV-1 provirus was demonstrated in skin lesions in one patient and in blood lymphocytes in the other. Chemotherapy, then interferon alpha, were unsuccessful in the first patient. Topical metchloretamine was partially effective for the second patient. DISCUSSION: ATL in siblings is explained by mother-to-child transmission of HTLV-1 infection during breastfeeding.

Adult↗
PubMed1997

Acquisition of granzyme B and Fas ligand proteins by human keratinocytes contributes to epidermal cell defense.

In vertebrate tissues, cell integrity is maintained by at least three mechanisms. During an immune response, injured cells are eliminated by cytotoxic lymphoid cells that produce perforin, granzyme B, and Fas ligand (FasL). Second, epithelial cells can produce FasL as an immunosuppressive protein, probably to protect the tissue against immune-mediated damage. Third, locally secreted antimicrobial peptides can be operative in the protection of animal and human epithelia. In this work, as another contribution to local mechanisms of host defense, the ability of human epidermal keratinocytes to produce cytotoxic proteins was investigated. To address this question, freshly isolated human epidermal cells and keratinocytes grown in vitro were studied. Freshly isolated epidermal cells did not express the cytolytic proteins. In contrast, keratinocyte growth to confluence was associated with granzyme B, perforin, and FasL mRNA and protein synthesis. These proteins were secreted in the culture medium. Further analysis showed that they were identical with the ones used by cytotoxic lymphocytes. Their function was then investigated with a view to a potential role in epidermal cell integrity. The data showed that activated human keratinocytes were able to protect against invading pathogens through granzyme B expression. This was demonstrated by the ability of granzyme B to greatly decrease the bacterial growth of Staphylococcus epidermidis. In addition, keratinocytes expressing FasL were found to prevent immune epidermal cell damage. Apoptosis of Fas-sensitive T cells occurred during coculture with confluent epidermal keratinocytes and was largely reduced by the addition of a FasL inhibitor. The data favor keratinocyte involvement in the regulation of dermal inflammatory responses.

Apoptosis↗
PubMed1997

Schönlein-Henoch purpura in adult patients. Predictive factors for IgA glomerulonephritis in a retrospective study of 57 cases.

OBJECTIVES: To evaluate the incidence of extracutaneous manifestations and to identify predictive factors for renal involvement in adult patients with Schönlein-Henoch purpura. DESIGN: Retrospective study with a comparative analysis of patients with and without renal involvement. SETTING: Patients who were attending the dermatologic department of an academic medical center. PATIENTS: In patients with purpura of the lower limbs and cutaneous vascular IgA deposits for which cases were recorded from 1985 to 1993, the following selection criteria were used: age older than 15 years and absence of thrombocytopenia, of IgA deposits in the basement membrane zone, and of a known hematologic or connective tissue disorder. MAIN OUTCOME MEASURES: Clinical and biological data, results of histological studies, and findings from direct immunofluorescence studies of skin biopsy specimens were compared in patients with and without renal involvement. RESULTS: Fifty-seven patients were included: 23% had an IgA glomerulonephritis confirmed by results of a renal biopsy, and a further 26% showed abnormalities on urine microscopy. Joint and gastrointestinal involvement was noted in, respectively, 33% and 19% of the patients. A comparative analysis of patients with and without renal involvement failed to reveal significant differences with regard to age, sex, the presence of bullous or necrotic cutaneous lesions, gastrointestinal or joint involvement, histological features, and findings from direct immunofluorescence studies. An IgA glomerulonephritis was significantly associated with purpura above the waist (P = .03), a recent infectious history (P = .02), pyrexia (P = .01), and biological markers of inflammation (P = .006). CONCLUSIONS: Despite a lower incidence of systemic involvement compared with that in other published series, the incidence of renal involvement remained high (ie, between 23% and 49%). A recent infectious history, pyrexia, the spread of purpura to the trunk, and biological markers of inflammation were predictive factors for renal involvement.

Adult↗
PubMed1997

Daily multidrug therapy for leprosy; results of a fourteen-year experience.

Between 1980 and 1994, 67 new or relapsing leprosy patients were treated by daily administered multidrug regimens. Tuberculoid patients (23 TT/BT) received either bitherapy [rifampin + dapsone or clofazimine (RMP + DDS or CLO)] or tritherapy [RMP + DDS and/or CLO and/or ethionamide (ETH)] until clinical cure. Lepromatous patients (44 BB/BL/LL) received tritherapy (RMP + DDS and/or CLO and/or ETH) at least until bacteriological negativity. Of the 23 tuberculoid patients only one patient (5%) was cured at 6 months and about 70% needed between 6 and 24 months of treatment to obtain clinical cure (mean 19.5 months). In the 44 lepromatous patients, the achievement of bacteriological negativity was significantly linked to the initial bacterial index (BI), and it occurred after 2 to 7 years (mean 66.5 months) of multidrug therapy (MDT). The average BI decrease per year was 1.1+ during the first year, 0.9+ the second year, and then < 0.5+ per year. Reactional states significantly (p < 0.01) influenced the BI course: reversal reactions (RR) accelerated while erythema nodosum leprosum (ENL) delayed the BI decrease. Three of the 23 (13%) tuberculoid and 19 of the 44 (43%) lepromatous patients (p < 0.02) exhibited a RR and 18 of 44 (41%) lepromatous patients had ENL during MDT. A late RR (LRR) was observed in 1 (5%) and 6 (17%) of our tuberculoid and lepromatous patients, respectively, and 3 (8%) of our lepromatous patients suffered post-MDT ENL. No confirmed relapse has been observed within a follow-up period of 6 months to 7 years and 3 months [59 person-years at risk (PYR)] for TT/BT patients and of 4 months to 5 years and 10 months (100 PYR) for BB/BL/LL patients. When compared to the recommended WHO/MDT, it appears that daily MDT does not increase the clinical or the bacteriological cure rates either at 6 months in paucibacillary tuberculoid patients or at 2d years in multibacillary lepromatous patients. Moreover, as does the WHO/MDT, our regimens show a high frequency of reactional states both during and after treatment. This fact constitutes the main new problem of the actual treatment of leprosy.

Adolescent↗
PubMed1996

[Urinary porphyrin excretion in human immunodeficiency virus infection].

OBJECTIVES: Porphyria cutanea tarda has been reported in about 60 patients with human immunodeficiency virus (HIV) since 1987. We looked for porphyrin metabolism disorders in HIV infected patients without patent porphyria cutanea tarda. METHODS: Urinary porphyrin excretion was measured in 64 patients with an HIV infection. RESULTS: Excreted levels were abnormal in 23 patients (36%) including 4 (6%) with patterns highly suggestive of porphyria cutanea tarda. One of these patients developed the disease 2 years later. In 15 patients (23%) there was a significant increase in coproporphyrinuria. Minimal alterations in uroporphyrin or its precursors were seen in the other patients. Abnormal excretion was significantly more frequent in patients with hepatopathy and in patients who had progressed to AIDS. CONCLUSION: Our findings suggest that HIV infection does not play a direct role in altered porphyrin metabolism because of the frequency of liver disorders observed in these patients.

Acquired Immunodeficiency Syndrome↗
PubMed1996

Cutaneous T-cell lymphoma associated with granulomatous slack skin.

Granulomatous slack skin disease (GSS) is a rare disorder characterized by bulky cutaneous lesions and epithelioid and giant cell granulomas with destruction of the dermal elastic tissue. We detail the observation of a 29-year-old man with clinical and histological features of GSS. Pendulous skin tumors were associated with typical clinical and immunohistochemical aspects of mycosis fungoides and with clonal rearrangement of the V gamma T-cell receptor gene in lesional skin. This case report supports cutaneous T-cell lymphoma as a cause of GSS.

Adult↗
PubMed1996

Pemphigus in Mali: a study of 30 cases.

Pemphigus has been largely studied in developed countries (North America and Europe) and in Brazil. In these geographical settings, pemphigus presents two very different epidemiological and clinical patterns. Little is known about pemphigus in other regions of the world, particularly in Africa. We report here a study of 30 cases of pemphigus observed in Bamako, Mali. Our data suggest that pemphigus in this area presents a distinctive pattern. Our cases of pemphigus were diagnosed on the basis of clinical, histological and direct immunofluorescence studies. We estimated the annual incidence in the Bamako region to be 0.29 cases per 100,000 inhabitants. There was no endemic focus in Mali. The disease was observed mainly in women (24 of 30; 80%), especially those older than 40 years (mean age, 46.7 years), and in the Fulani ethnic group (10 of 30; 33%). Our study group was composed of 25 cases of pemphigus foliaceus (PF) (83%), four cases of pemphigus vulgaris and one case of pemphigus vegetans. Pustules with hypopyon were observed in 11 patients (37%). A diffuse verrucous change in the skin was noted in four cases of erythrodermic PF. In 16 patients with PF, localized verrucous lesions mimicking seborrhoeic keratoses were observed when oral corticosteroid treatment was decreased. Histopathological examination demonstrated eosinophilic spongiosis in 50% of patients. These data suggest that pemphigus in Mali differs from the two main known patterns of the disease: the North American/European one, and the Brazilian pattern, with which it shares the predominance of superficial forms but otherwise differs in many features.

Adult↗
PubMed1996

Immunohistochemical and virological study of skin in the papular-purpuric gloves and socks syndrome.

The pathogenesis of viral exanthems remains unclear. We have undertaken an immunohistochemical study of lesional skin biopsies in patients with the papular-purpuric gloves and socks syndrome (PPGSS) secondary to parvovirus B19 infection. Intracytoplasmic staining of the dermal endothelial cells, keratinocytes, and sweat glands was shown with an antibody to parvovirus B19. There were perivascular dermal infiltrates with T cells, sometimes with exocytosis. By polymerase chain reaction, virus DNA was detected in all skin biopsies and in one serum sample. The cutaneous manifestations of parvovirus infection seem secondary to infection of the endothelium and epidermis.

Adolescent↗
PubMed1996

[Bullous IgA linear dermatosis of children in Mali].

INTRODUCTION: Bullous IgA linear dermatosis is relatively frequent in children in Africa. The aim of this study was to evaluate the frequency of this disease among autoimmune bullous diseases in children in Mali. PATIENTS AND METHODS: Children with chronic bullous disease seen at the Marchoux Institute in Bamako and for whom direct immunofluorescence of the perilesional skin demonstrated immunoglobulin and/or complement deposits were included in the study population. RESULTS: Twelve children were included. Direct immunofluorescence showed linear IgA deposit along the dermo-epidermal junction in 10, once with a pemphigoid aspect and once with a pemphigus aspect. The skin lesions in the children with linear IgA deposits were stereotypic: disseminated bullae, rosettes (9 out of 10), involving the external genital organs (10/10), the limbs, the face and the trunk. Histology showed subepidermal bullae (10/10), with neutrophil papillar abscesses (9/10). Indirect immunofluorescence of the cleaved skin NaCl in 5 patients showed that the cleavage always lied at the roof of the cleavage. All the children responded to dapsone (1 to 2.5 mg/kg/d) which gave complete (7/10), or incomplete remission. Local corticosteroids were used in 2 patients and general corticosteroids in 1. DISCUSSION: This confirms the frequency of bullous IgA linear dermatosis in Africa, in contrast with uncommon dermatitis herpetiformis. Diagnosis is facilitated with immunofluorescence. The low cost of this test makes it a useful diagnostic tool in these countries.

Autoimmune Diseases↗