Clinical features, treatment options and outcomes in primary cutaneous B-cell lymphomas: a real-world, multicenter, retrospective study.

Background: Primary cutaneous B-cell lymphomas (PCBCLs) are rare cutaneous neoplasms with limited literature regarding treatment options and associated treatment outcomes. This study aimed to investigate and present real-world treatment outcomes in patients with PCBCLs.

Methods: All patients with PCBCL who were treated in five major referral centers for cutaneous lymphoma in Greece over 10 years were retrospectively included with their baseline characteristics and treatment-associated outcomes collected and analyzed.

The Impact of Immune Checkpoint Inhibitors-Induced Skin Toxicity on Patients Quality of Life and the Role of Dermatologic Intervention.

Introduction: Data regarding quality of life (QoL) of oncologic patients experiencing dermatologic immune-related adverse events (dirAEs) and their course after dermatologic intervention are scarce.

Objectives: To assess the impact of dirAEs on patients QoL and to investigate the correlation between dermatologic and oncologic indexes used for estimating QoL.

Methods: We enrolled oncologic patients with dirAEs managed in two supportive onco-dermatology outpatient clinics in Greece.

A Real-Life 208 Week Single-Centred, Register-Based Retrospective Study Assessing Secukinumab Survival and Long-Term Efficacy and Safety Among Greek Patients with Moderate to Severe Plaque Psoriasis, Including Difficult-to-Treat Manifestations Such as Genitals and Scalp.

Introduction: Psoriasis is a chronic inflammatory disease with multiple skin manifestations, and in case of lesions affecting the genital area, sexual health impairment and psychological distress can furthermore impair the patients quality of life. Secukinumab is a fully humanized immunoglobulin G1 kappa antagonist of IL-17A and is indicated for the treatment of moderate-to-severe psoriasis, since it shows a significant efficacy in clinical outcomes, with rapid onset of remission, prolonged treatment response rate, advantageous safety profile and a valuable improvement of the patients quality of life.

Objectives: This study was conducted in order to gather retrospective real-world data regarding the efficacy of secukinumab in treating patients with moderate-to-severe plaque psoriasis in Greece.

Efficacy and Safety of Rilzabrutinib in Pemphigus: PEGASUS Phase 3 Randomized Study.

Trial Design: Pemphigus is a rare but life-threatening autoimmune disease requiring long-term treatment that minimizes corticosteroid (CS) exposure while providing consistent disease control. The phase 2 pemphigus study of oral, reversible, covalent Bruton tyrosine kinase inhibitor rilzabrutinib demonstrated rapid and sustained efficacy with well-tolerated safety.

Methods: Adults (aged 18-80 years) were randomized 1:1 to 400 mg rilzabrutinib (n = 65) or placebo (n = 66) twice daily (with CS ≤ 0.

Differences among Indian and European pemphigus patients based on demographics, clinical parameters and propensity for relapse: results of a prospective bicontinental cohort study.

Limited studies have explored pemphigus variations among different ethnic groups residing in their respective geographical locations. This bicontinental study aimed to compare clinical and immunological parameters in Indian and European pemphigus patients in complete remission, off therapy, or on minimal therapy. 105 patients (India, n= 75; Bulgaria, n=15; Greece, n=15) with pemphigus vulgaris (PV) or pemphigus foliaceous (PF) in complete remission on minimal therapy (n=64) or complete remission off therapy (n=41) were recruited.

Efficacy of biologic agents for palmoplantar psoriasis: a systematic review and network meta-analysis.

Background: Palmoplantar psoriasis (PP) represents a localized type of disease. While controversy over its' classification exists, a hyperkeratotic type, a pustular type and palmoplantar pustulosis (PPP) have been recognized. PP management is regularly supported by biologic agents.

Eccrine Poroma: Pathogenesis, New Diagnostic Tools and Association with Porocarcinoma-A Review.

Eccrine poroma (EP) is a relatively rare benign adnexal neoplasm that usually affects elderly patients. Its pathogenesis is still under investigation, but recent gene studies have revealed gene fusions as key incidences resulting in oncogenetic pathways. It often presents as a solitary, firm papule, mostly asymptomatic, located on the soles or palms.

Clinical and Dermoscopic Characteristics of Cutaneous Sarcomas: A Literature Review.

Under the umbrella of cutaneous sarcomas (CS) we include a heterogeneous group of rare, malignant, mesenchymal neoplasia, such as dermatofibrosarcoma protuberans, atypical fibroxanthoma, cutaneous undifferentiated pleomorphic sarcoma, cutaneous angiosarcoma and leiomyosarcoma. Clinical presentation and histopathological examination are the cornerstone of CS diagnosis and classification. There are scarce data in the literature in regards to the clinical and dermatoscopic characteristics of CS and the role of dermatoscopy in their early identification.

S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV).

Background: Paraneoplastic pemphigus (PNP), also called paraneoplastic autoimmune multiorgan syndrome (PAMS), is a rare autoimmune disease with mucocutaneous and multi-organ involvement. PNP/PAMS is typically associated with lymphoproliferative or haematological malignancies, and less frequently with solid malignancies. The mortality rate of PNP/PAMS is elevated owing to the increased risk of severe infections and disease-associated complications, such as bronchiolitis obliterans.

Narrative Review of Drug-Associated Nail Toxicities in Oncologic Patients.

Introduction: Nail toxicity represents one of the most common cutaneous adverse effects of both classic chemotherapeutic agents and new oncologic drugs, including targeted treatments and immunotherapy.

Objectives: We aimed to provide a comprehensive literature review of nail toxicities derived from conventional chemotherapeutic agents, targeted therapies (EGFR inhibitors, multikinase inhibitors, BRAF and MEK inhibitors) and immune checkpoint inhibitors (ICIs), including clinical presentation, implicated drugs and approaches for prevention and management.

Methods: Retrieved literature from PubMed registry database was reviewed to include all articles published up to May 2021 relevant to the clinical presentation, diagnosis, incidence, prevention, and treatment of oncologic treatment-induced nail toxicity.

Restrictive dermopathy due to ZMPSTE24 deficiency.

Restrictive dermopathy (RD) (OMIM 275210) is a rare, lethal genodermatosis belonging to the group of laminopathies. It is caused by biallelic variants in , which is involved in lamin A post-translational processing or, less frequently, by monoallelic variants in , leading to accumulation of truncated prelamin A protein (Navarro et al., 2004; Navarro et al.

Pharmacogenetic Analysis of the rs2910164 and rs767649 Polymorphisms and Response to Anti-TNF Treatment in Patients with Crohn's Disease and Psoriasis.

The clinical heterogeneity regarding the response profile of the antitumor necrosis factor (anti-TNF) in patients with Crohn's disease (CD) and psoriasis (PsO) is attributed, amongst others, to genetic factors that influence the regulatory mechanisms which orchestrate the inflammatory response. Here, we investigated the possible associations between the rs2910164 and rs767649 variants and the response to anti-TNF therapy in a Greek cohort of 103 CD and 100 PsO patients. We genotyped 103 CD patients and 100 PsO patients via the PCR-RFLP method, utilizing the de novo formation of a restriction site for the SacI enzyme considering the rs2910164, while Tsp45I was employed for the rs767649 variant.

Dysregulation of Plasma miR-146a and miR-155 Expression Profile in Mycosis Fungoides Is Associated with rs2910164 and rs767649 Polymorphisms.

Diagnosis of Mycosis Fungoides (MF) may be challenging, due to its polymorphic nature. The use of miRNAs as biomarkers to assist in diagnosis has been investigated, mainly in skin lesion biopsies. The purpose of this study is to evaluate the plasma levels of miR-146a and miR-155 in MF patients and to investigate their association with SNPs of their genes.