Repair of the lower and middle parts of the face by composite tissue allotransplantation in a patient with massive plexiform neurofibroma: a 1-year follow-up study.

Background: The risk to benefit ratio of face transplantation with a composite tissue allograft remains debatable, although this procedure is technically feasible. We report here a 1-year follow-up of a patient who underwent face transplantation with a composite tissue allograft.

Methods: On Jan 21, 2007, a 29-year-old man with neurofibromatosis type 1 underwent resection of a massive plexiform neurofibroma diffusely infiltrating the middle and lower part of his face.

Histopathologically dysplastic neurofibromas in neurofibromatosis 1: diagnostic criteria, prevalence and clinical significance.

Background: Malignant peripheral nerve sheath tumours (MPNSTs) correspond to the most frequent and aggressive neoplasic complications associated with poor prognosis in neurofibromatosis 1.

Objectives: To define the dysplastic neurofibroma potentially at risk of transformation and evaluate its prevalence and incidence.

Methods: According to our database, we retrospectively included, between 1 March 2000 and 31 August 2004, all patients who had subcutaneous and/or plexiform neurofibromas removed surgically.

SOX9 expression is a general marker of basal cell carcinoma and adnexal-related neoplasms.

Background: SOX9 is a transcription factor that fulfills multiple functions during development. In the hair follicle SOX9 is expressed in the outer layer of the epithelial sheath, and the hair stem cell compartment. Recent data suggest that Sox9 acts as a downstream target of the Sonic hedgehog (Shh) pathway.

CD158K/KIR3DL2 transcript detection in lesional skin of patients with erythroderma is a tool for the diagnosis of Sézary syndrome.

The distinction between Sézary syndrome (SS) and benign erythrodermic inflammatory diseases (EID) is difficult to make both clinically and on skin biopsies, since histomorphology can provide nonspecific results. New markers of circulating malignant Sézary cells have been recently described, especially CD158k/KIR3DL2 and T-plastin, but it has not been yet determined whether they could help in the diagnosis of erythroderma in skin samples. In this study, 13 frozen skin specimens from 10 SS patients and 26 from EID were analyzed for CD158k/KIR3DL2 expression using immunohistochemistry with AZ158 mAb, which also recognizes the monomeric CD158e/KIR3DL1 receptor.

Characterization of CXCL13+ neoplastic t cells in cutaneous lesions of angioimmunoblastic T-cell lymphoma (AITL).

Skin manifestations of angioimmunoblastic T-cell lymphoma (AITL) are frequent, sometimes as first manifestations of the disease. In the absence of a specific marker for neoplastic cells, diagnosis of AITL in skin biopsies is often difficult. CD10 and CXCL13 have been recently recognized as characteristic markers of AITL, but have not been yet investigated in the skin.

Study of the reactive dendritic cells in small B-cell lymphoproliferations of the skin.

Distinguishing between low-grade primary cutaneous B-cell lymphoma (LG-pCBCL) and cutaneous lymphoid hyperplasia (CLH) based on histological features is often difficult. CLH lesions contain numerous reactive cells of the histiocyte lineage [Langerhans cells (LC), dermal dendritic cells (DDC), and macrophages], which are also often present in CBCL. The aim of this study was to determine whether immunohistochemical detection of those cells could help differentiate between CLH and LG-pCBCL.

Identification of a novel CD160+ CD4+ T-lymphocyte subset in the skin: a possible role for CD160 in skin inflammation.

CD160 is a glycosylphosphatidylinositol-anchored cell surface molecule expressed by human circulating cytotoxic lymphocytes that correspond to the majority of natural killer cell (NK) expressing CD56(dim), TCRgammadelta lymphocytes, and to a minor CD8 T-cell subset. CD160 engagement by major histocompatibility complex class I molecules triggers by itself both cytotoxic function and cytokine production in NK lymphocytes, whereas it provides co-activating signals to TCR-induced proliferation in T CD8+ lymphocytes. In this study, we analyzed by immunohistochemistry the phenotype of lymphocytes infiltrating normal skin and inflammatory skin lesions of atopic dermatitis, contact dermatitis, and psoriasis.

Microarray-based identification of tenascin C and tenascin XB, genes possibly involved in tumorigenesis associated with neurofibromatosis type 1.

Purpose: Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder with a complex variety of clinical manifestations. The hallmark of NF1 is the onset of heterogeneous (dermal or plexiform) benign neurofibromas. Plexiform neurofibromas can give rise to malignant peripheral nerve sheath tumors, which are resistant to conventional therapies.

[Choroidal white dots and sarcoidosis].

Introduction: Sarcoidosis is a multisystemic disease characterized by a noncaseating granuloma. Posterior segment disease with white choroidal dots occurs in only 5% of sarcoidosis cases with ocular involvement.

Observation: We report here the case of a 45-year-old Caucasian patient complaining of a skin rash.

Significance of circulating T-cell clones in Sezary syndrome.

Identification of malignant Sézary cells by T-cell receptor (TCR) clonality studies is routinely used for the diagnosis of Sézary syndrome, but T-cell clones expressed in a single patient have never been accurately characterized. We previously reported that CD158k expression delineates Sézary syndrome malignant cells, and, more recently, we identified vimentin at the surface membranes of Sézary cells and normal activated lymphocytes. In the present study, T-cell clones from 13 patients with Sézary syndrome were identified by immunoscopy and further characterized in the blood according to their TCR Vbeta, CD158k, and vimentin cell-surface expression.

Circulating natural killer lymphocytes are potential cytotoxic effectors against autologous malignant cells in sezary syndrome patients.

Patients with advanced cutaneous T cell lymphoma (CTCL) exhibit profound defects in cell-mediated immunity. Although it has been suggested that Sezary syndrome (SS) patients have a decreased natural killer (NK) lymphocyte activity, nothing has been reported concerning the sensitivity of Sezary cells to NK lymphocyte-mediated cytotoxicity. Peripheral blood NK cells from healthy donors were tested against Sezary tumoral cell lines as well as against freshly isolated Sezary cells.

Granuloma faciale: a clinicopathologic study of 66 patients.

Background: Only case reports or studies of small series of patients have focused on granuloma faciale (GF).

Objective: We sought to describe the clinicopathologic characteristics of GF in a large series of patients.

Methods: We conducted a retrospective analysis of 66 patients and 73 skin specimens.

Sox9 is essential for outer root sheath differentiation and the formation of the hair stem cell compartment.

Background: The mammalian hair represents an unparalleled model system to understand both developmental processes and stem cell biology. The hair follicle consists of several concentric epithelial sheaths with the outer root sheath (ORS) forming the outermost layer. Functionally, the ORS has been implicated in the migration of hair stem cells from the stem cell niche toward the hair bulb.

Mucosa-associated lymphoid tissue lymphoma of the thymus: a case report with no evidence of MALT1 rearrangement.

We report a case of thymic mucosa-associated lymphoid tissue (MALT) lymphoma (TML) that presented as an asymptomatic mediastinal mass in a 40-year-old woman with a past history of Sjögren syndrome. This case had the characteristic clinical and pathological features of TML, as found in most of the 24 previously reported cases, i.e.