The Microenvironment's Role in Mycosis Fungoides and Sézary Syndrome: From Progression to Therapeutic Implications.

Background: Mycosis fungoides (MF) and Sezary Syndrome (SS) are the most common cutaneous T-cell lymphomas. It has been hypothesized that the interaction between the immune system, cutaneous cells, and neoplastic elements may play a role in MF/SS pathogenesis and progression.

Methods: This paper aims to revise in a narrative way our current knowledge of the microenvironment's role in MF/SS.

Newly-Discovered Neural Features Expand the Pathobiological Knowledge of Blastic Plasmacytoid Dendritic Cell Neoplasm.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and highly aggressive hematologic malignancy originating from plasmacytoid dendritic cells (pDCs). The microRNA expression profile of BPDCN was compared to that of normal pDCs and the impact of miRNA dysregulation on the BPDCN transcriptional program was assessed. MiRNA and gene expression profiling data were integrated to obtain the BPDCN miRNA-regulatory network.

Deregulation of JAK2 signaling underlies primary cutaneous CD8 aggressive epidermotropic cytotoxic T-cell lymphoma.

Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma (pcAECyTCL) is a rare variant of cutaneous T-cell lymphoma with an aggressive clinical course and a very poor prognosis. Until now, neither a systematic characterization of genetic alterations driving pcAECyTCL has been performed, nor effective therapeutic regimes for patients have been defined. Here, we present the first highresolution genetic characterization of pcAECyTCL by using wholegenome and RNA sequencing.

Whole-genome analysis uncovers recurrent IKZF1 inactivation and aberrant cell adhesion in blastic plasmacytoid dendritic cell neoplasm.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and highly aggressive hematological malignancy with a poorly understood pathobiology and no effective therapeutic options. Despite a few recurrent genetic defects (eg, single nucleotide changes, indels, large chromosomal aberrations) have been identified in BPDCN, none are disease-specific, and more importantly, none explain its genesis or clinical behavior. In this study, we performed the first high resolution whole-genome analysis of BPDCN with a special focus on structural genomic alterations by using whole-genome sequencing and RNA sequencing.

Linear IgA bullous dermatosis in adults and children: a clinical and immunopathological study of 38 patients.

Background: Linear IgA bullous dermatosis (LABD) is a rare autoimmune subepithelial vesiculobullous disease due to IgA autoantibodies directed against different antigens of the basement membrane zone (BMZ) of the skin and/or mucosae. It affects mainly preschool-aged children and adults, with only few studies on large series. The aim of this study was to assess possible differences between adults and children regarding clinical presentation, immunopathologic features, management and course of the disease.

CD30 expression in a case of intravascular large B-cell lymphoma, cutaneous variant.

Intravascular large B-cell lymphoma (IVLBCL) is one of the rarest B-cell non-Hodgkin lymphomas (NHL), with an aggressive clinical behavior and a poor prognosis; in fact, its treatment is still an unmet clinical need, with a 3-year overall survival (OS) rate of 60% to 81%, and a central nervous system relapse rate of 25%. It usually presents as a widespread disease at diagnosis, with multi-organ involvement. Previously considered as a diffuse large B-cell lymphoma variant, it now represents a different extranodal large B-cell lymphoma entity in the last WHO Classification of tumors of hematopoietic and lymphoid tissues.

Array-based CGH of primary cutaneous CD8+ aggressive EPIDERMO-tropic cytotoxic T-cell lymphoma.

Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma (pcAECyTCL) is a rare provisionally categorized cutaneous lymphoma characterized by an aggressive course. Its pathogenesis and molecular mechanisms are still unknown, and only two individual cases have so far been molecularly characterized. The aim of this study was to define the pattern of numerical chromosomal alterations in tumor samples taken from 20 patients with pcAECyTCL at the time of diagnosis by means of array-comparative genomic hybridization (a-CGH).

Detection of IgE autoantibodies in mucous membrane pemphigoid and their association with disease severity.

Background: Mucous membrane pemphigoid (MMP) is an autoimmune disease characterized by scarring lesions at mucosal sites. Although the pathogenic role of specific IgG and/or IgA has been already demonstrated and the detection of these immunoglobulins is a criterion in the diagnosis of MMP, little is known about IgE role in this disease. Therefore, the main purpose of this study was to assess the presence of circulating and tissue bound IgE in patients with MMP and their possible correlations with clinical presentation and disease course.

Immunohistochemical expression and prognostic role of CD10, CD271 and Nestin in primary and recurrent cutaneous melanoma.

Background: CD10, CD271 and Nestin, which are proteins associated with tumor-initiating properties and/or progression potential, have not been specifically studied on malignant melanoma (MM) with cutaneous recurrences.

Methods: We evaluated the expression of CD10, CD271 and Nestin in 27 tumor samples from 16 patients. These tumor samples corresponded to 6 primary melanomas which developed 11 ITM and 10 primary melanomas without recurrences at 10-year follow-up from specimens obtained from surgical excisions of patients referred to the Unit of Dermatology, Department of Medical-Surgical and Transplant Physiopathology, University of Milan, between 2006 and 2016.

Nestin Expression in Spitzoid Lesions: An Immunohistochemical Characterization With Clinical and Dermoscopic Correlations.

Spindle or epithelioid melanocytic (Spitz) nevi usually affect children or adolescents and growth in the face or the lower extremities. Histologically, they may show cytoarchitectural atypia and mitotic figures that could represent diagnostic pitfalls with malignant melanoma. Atypical spitzoid tumors (AST) indicate lesions that microscopically show intermediate characteristics between benign nevi and malignant melanoma.

Cutaneous metastases of internal malignancies: an experience from a single institution.

Background: Cutaneous metastases represent 2% of all skin tumours. Their recognition can be challenging, as they may present with different clinical features, with consequent frequent delay and failure in diagnosis.

Objectives: To review our series of cutaneous metastatic lesions, analyse their frequency according to patient gender, histotype, localization of the primary tumour, and site of cutaneous metastasis, and correlate this data with clinicopathological parameters.

Primary cutaneous acral CD8 positive T-cell lymphoma with extra-cutaneous involvement: A long-standing case with an unexpected progression.

Primary cutaneous acral CD8+ T-cell lymphoma (acral CD8+ TCL) is a new provisional entity characterized by acral skin lesions and an indolent course. We describe an extraordinary case characterized by relapsed nodules with CD8+ cytotoxic infiltrates on the left ear. After 35 years, the skin lesions spread to other acral sites, and a mass with the same histological features as the other skin lesions appeared on the nose.

Erythema multiforme-like lesions in primary cutaneous aggressive cytotoxic epidermotropic CD8+ T-cell lymphoma: A diagnostic and therapeutic challenge.

Primary cutaneous aggressive cytotoxic epidermotropic CD8+ T-cell lymphoma is an extremely rare, rapidly progressing, cutaneous lymphoma, with frequent systemic involvement and poor prognosis, that still represents a diagnostic and therapeutic challenge, especially in the early stage. Herein, we report a case of an elderly woman with a fulminant course, who at onset presented with clinical and pathological features mimicking erythema multiforme (EM) and treated with cyclosporine that led to rapid deterioration with fatal outcome 6 months after disease onset. Histopathology showed a lichenoid, epidermotropic and nodular, angiocentric, dermal and subcutaneous infiltrate of sF1, CD8+, CD45RA+ small to medium-sized atypical lymphoid cells, which strongly expressed cytotoxic markers.

Clinicopathological and molecular study of primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma.

Background: Primary cutaneous CD4+ small-/medium-sized pleomorphic T-cell lymphoma (CD4+ PCSM-TCL) is a rare lymphoproliferative disorder with a favorable prognosis. Distinguishing it from other cutaneous lymphomas is often a challenge.

Methods: We retrospectively collected CD4+PCSM-TCL cases from two centers (MD Anderson Cancer Center, USA and University of Milan, Italy) and evaluated their clinicopathological features.

Sézary Syndrome in a 17-Year-Old Boy: Clinicopathologic Features and Genomic Profile.

We describe the case of a 17-year-old Hispanic boy who had had erythroderma and diffuse lymphadenopathy for approximately 6 months. A diagnosis of Sézary syndrome was made on the basis of the histologic features of the skin; the presence of the same T-cell clone on the skin, blood, and bone marrow; and the high CD4(+) lymphocyte count with an aberrant phenotype in peripheral blood; bone marrow involvement was also present. The patient was treated with systemic gemcitabine and achieved partial remission.

Rash caused by Oryctes nasicornis.

We report a case of rash caused by crushing of a male of Oryctes nasicornis (Linnaeus 1758) (Coleoptera, "http:// it. wikipedia. org/ wiki/ Scarabaeidae" \o "Scarabaeidae" Scarabaeidae), popularly known as "European rhinoceros beetle", on the skin of an Italian tourist who developed the reaction during a trip to Turkey.

Association of pyoderma gangrenosum, acne, and suppurative hidradenitis (PASH) shares genetic and cytokine profiles with other autoinflammatory diseases.

The association of pyoderma gangrenosum, acne, and suppurative hidradenitis (PASH) has recently been described and suggested to be a new entity within the spectrum of autoinflammatory syndromes, which are characterized by recurrent episodes of sterile inflammation, without circulating autoantibodies and autoreactive T-cells. We conducted an observational study on 5 patients with PASH syndrome, analyzing their clinical features, genetic profile of 10 genes already known to be involved in autoinflammatory diseases (AIDs), and cytokine expression pattern both in lesional skin and serum. In tissue skin samples, the expressions of interleukin (IL)-1β and its receptors I and II were significantly higher in PASH (P = 0.