F Cambazard

Publications (7)13.6 Total impact

• Article: A longitudinal study of a harlequin infant presenting clinically as non-bullous congenital ichthyosiform erythroderma.

  M Haftek, F Cambazard, D Dhouailly, A Réano, M Simon, A Lachaux, G Serre, A Claudy, D Schmitt
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  ABSTRACT: Over the past 8 years, we have followed a child born as a harlequin baby, who survived due to treatment with retinoids. His condition evolved clinically towards the erythrodermic form of lamellar ichthyosis (non-bullous congenital ichthyosiform erythroderma, NBCIE). According to ultrastructural and biochemical criteria, our patient originally presented with type II harlequin ichthyosis. Investigations showed an abnormal keratinosome structure and extrusion, a keratin pattern characteristic for epidermal hyperproliferation, and an absence of conversion of profilaggrin to filaggrin. Persisting keratinocyte hyperproliferation, associated with the presence of a dermal infiltrate, is in agreement with the present clinical picture of severe NBCIE. However, abnormal lamellar body production and defective filaggrin processing, which is not one of the diagnostic criteria of NBCIE, persist in the patient's skin. Further studies of the epidermal lipid composition, and of possible mutations of the keratinocyte transglutaminase gene performed on epidermal cell cultures of harlequin ichthyosis, will be necessary before type II harlequin ichthyosis can be accepted as an extremely severe form of NBCIE.
  British Journal of Dermatology 10/1996; 135(3):448-53. · 3.67 Impact Factor
• Article: Eosinophilic granuloma of bone and biochemical demonstration of 49-kDa CD1a molecule expression by Langerhans-cell histiocytosis.

  F Cambazard, C Dezutter-Dambuyant, M J Staquet, D Schmitt, J Thivolet
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  ABSTRACT: Histiocytic cells infiltrating the lesions in eosinophilic granuloma of bone as well as in cutaneous histiocytosis X were studied using a murine monoclonal antibody (MA) produced with proliferating cells from an eosinophilic granuloma of bone. This MA reacts with Langerhans cells (LC) of normal human skin or mucous membranes and with proliferating cells of eosinophilic granuloma of bone and skin lesions of Letter-Siwe disease, as shown by immunohistochemistry and immunogold labelling. As other murine MA's obtained after immunization with human cortical thymocytes, this MA immunoprecipitates the 49-kDa CD1a antigen found on human LC and thymic-cell surfaces but not its breakdown product after treatment with trypsin, as demonstrated by analysis of immunoelectron labelling, cytofluorometry and gel electrophoresis. This first production of a CD1a MA from an eosinophilic granuloma supports the concept of Langerhans-cell histiocytosis.
  Clinical and Experimental Dermatology 10/1991; 16(5):377-82. · 1.20 Impact Factor
• Article: Monoclonal antibody GB3 defines a widespread defect of several basement membranes and a keratinocyte dysfunction in patients with lethal junctional epidermolysis bullosa.

  P Verrando, C Blanchet-Bardon, A Pisani, L Thomas, F Cambazard, R A Eady, O Schofield, J P Ortonne
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  ABSTRACT: An antigen expressed at the dermal-epidermal junction as well as in some other human basement membranes (BM) has been detected by the use of a monoclonal antibody termed GB3. This antigen, synthesized by cultured normal human keratinocytes, has been identified as a 600-kilodalton glycoprotein different from other known components of BM. Using indirect immunofluorescence, GB3 was found to be not reactive with the epidermal BM in patients with lethal junctional epidermolysis bullosa. The present study demonstrates (by indirect immunofluorescence) that GB3 defines a widespread defect of several BM in these patients. Furthermore, it gives evidences for an intrinsic biologic defect of lethal junctional epidermolysis bullosa epidermal keratinocytes using in vitro culture of these cells. Whether the lack of GB3 reactivity is the consequence of a true absence of the antigen or an alteration of its molecular structure is not yet known. Nevertheless, GB3 is a useful probe for both rapid and prenatal diagnosis of lethal junctional epidermolysis bullosa, which will give new insights into the molecular comprehension of this disorder.
  Laboratory Investigation 02/1991; 64(1):85-92. · 3.64 Impact Factor
• Article: Cultured epithelia from junctional epidermolysis bullosa letalis keratinocytes express the main phenotypic characteristics of the disease.

  L Thomas, M Faure, F Cambazard, J Kanitakis, P Verrando, J P Ortonne, J Thivolet
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  ABSTRACT: Keratinocytes from a 1-week-old male infant with junctional epidermolysis bullosa letalis (JEBL) were grown in vitro and then grafted as multi-layered epithelia onto nude mice, to investigate whether the defect in the dermo-epidermal cohesiveness in the disease is of epidermal and not mesodermal origin. In culture, there was a birefringent ring of cells at the edges of the keratinocyte colonies and in places some cells looked as though they had been ejected from the periphery of the colony. At confluence, the multi-layered epithelia were easily detached from the culture flasks using only mechanical agitation. On microscopy the fully-differentiated epithelium on days 21, 30 and 40 after grafting sometimes showed blistering at the dermal-epidermal junction. No labelling was noted using a GB3 monoclonal antibody, that reacts with normal human keratinocytes in culture and with the dermo-epidermal basement membrane zone in normal skin. This indicates that the defect of JEBL may be reproduced in culture and also after grafting the cultured epithelial onto a wound without an epidermis. This suggests a possible role for the junctional structure recognized by GB3 in dermo-epidermal cohesiveness.
  British Journal of Dermatology 03/1990; 122(2):137-45. · 3.67 Impact Factor
• Article: DMC1: a monoclonal antibody produced from histiocytosis X cells which reacts with the native CD1a molecule of human epidermal Langerhans cells.

  C Dezutter-Dambuyant, D Schmitt, M J Staquet, M Gaucherand, F Cambazard, J Thivolet
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  ABSTRACT: Human epidermal Langerhans cells express two (CD1a and CD1c) of the three human thymic cell surface differentiation antigens (CD1a, CD1b, and CD1c). The first cluster of differentiation antigens (CD1) is defined by a group of monoclonal antibodies (MCA). All these MCA were obtained after immunization of mice or rats with human cortical thymocytes. OKT6 MCA (a CD1a MCA) was the first to be described as reactive with human epidermal Langerhans cells. We produced a murine MCA, called DMC1, after immunization with proliferating Langerhans cells of Eosinophilic Granuloma of the bone (Histiocytosis X). In tissues DMC1 MCA reacted with epidermal dendritic cells (Langerhans cells) in the skin and cortical thymocytes in the thymus as observed on indirect immunofluorescence. At the ultrastructural level, DMC1 MCA was specific for Birbeck granule-containing Langerhans cells and did not react with melanocyte and keratinocyte populations. The quantitative analysis of immunoelectron labeling and the cytofluorometric study showed that the intensity of labeling was inversely correlated with the concentration of trypsin used in the preparation of epidermal cell from skin samples. DMC1 MCA precipitated a protein with a relative mass of 49,000 (CD1a molecule) from lysates of iodinated epidermal Langerhans cells under reducing conditions. It recognized the original CD1a molecule (Mr 49,000) but not the membrane breakdown product of CD1a (Mr 27,000) brought about by trypsin.
  Hybridoma 05/1989; 8(2):199-208.
• Article: [Bullous scleroderma with the histological appearance of lichen sclerosus et atrophicus].

  J F Nicolas, P Perret-Liaudet, C Adam, J Kanitakis, F Cambazard, C Hermier, J Thivolet
  Annales de Dermatologie et de Vénéréologie 02/1988; 115(4):461-6. · 0.72 Impact Factor
• Article: [Harlequin fetus treated with etretin (RO 10-1670)].

  F Cambazard, M Haftek, C Hermier, A Lachaud, J Dutruge, S Gallet, J P Armand, M J Staquet, J Thivolet
  Annales de Dermatologie et de Vénéréologie 02/1988; 115(11):1128-30. · 0.72 Impact Factor