Immune-mediated inflammatory diseases with skin manifestations (skIMIDs) may affect multiple organs. Interdisciplinary care (IC) can overcome the fragmented knowledge used in the management of skIMIDs. In skIMIDs-care, patient and healthcare practitioner (HCP) satisfaction with IC is unknown.
View Article and Find Full Text PDFActa Dermatovenerol Alp Pannonica Adriat
December 2023
Autoimmune blistering skin diseases (AIBDs) encompass several heterogeneous conditions clinically characterized by blisters and erosions on the skin and mucous membranes and are immunopathologically characterized by autoantibodies against structural proteins of the skin. Those proteins are responsible for the intercellular contact between epidermal keratinocytes and adhesion of the basal keratinocytes to the dermis. Therefore, AIBDs are divided into two main groups: intraepidermal (the pemphigus group) and subepidermal (the pemphigoid) groups.
View Article and Find Full Text PDFGenetic aspects have a substantial role in hidradenitis suppurativa (HS) pathogenesis. A positive family history of HS occurs in about one-third of HS cases and is significantly higher in patients with early onset of the disease. Recent twin studies have shown a high heritability in HS, fortifying the importance of genetic factors in disease pathogenesis.
View Article and Find Full Text PDFPemphigus vulgaris (PV) is an acquired autoimmune blistering disease characterized by the production of autoantibodies targeting desmosomal cadherins, primarily desmoglein 1 and desmoglein 3, leading to acantholysis. The etiology of PV is multifactorial, including genetic susceptibility. This retrospective study aimed to evaluate the association of HLA class II alleles and PV and to examine the impact of PV-associated HLA class II alleles on the concentration of anti-desmoglein antibodies.
View Article and Find Full Text PDFBackground: 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.
View Article and Find Full Text PDFDuring recent decades, the number of patients diagnosed with cancer has been increasing. Conventional treatments, which comprise chemotherapy, radiotherapy, surgery, and hormonal treatment, represent improvements in effectiveness and safety of administration and continue to be the standard model of treating malignancies. Advances in oncology have enabled the development of newer therapies such as immunotherapy and targeted therapy.
View Article and Find Full Text PDFEpidermolysis bullosa acquisita (EBA) is a rare chronic autoimmune subepidermal blistering disease of the skin and mucous membranes, usually beginning in adulthood. EBA is induced by autoantibodies to type VII collagen, a major component of anchoring fibrils in the dermal-epidermal junction (DEJ). The binding of autoantibodies to type-VII collagen subsequently leads to the detachment of the epidermis and the formation of mucocutaneous blisters.
View Article and Find Full Text PDFBackground: Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease of the skin and mucous membranes. This disease typically affects the elderly and presents with itch and localized or, most frequently, generalized bullous lesions. A subset of patients only develops excoriations, prurigo-like lesions, and eczematous and/or urticarial erythematous lesions.
View Article and Find Full Text PDFPemphigus is a rare autoimmune disease characterized by the production of pathogenic autoantibodies against desmosomal adhesion proteins, desmoglein 1 and 3. The pathophysiological process leads to the development of blisters and erosions on mucosal and/or skin surfaces as the main clinical manifestation of the disease. Rituximab emerged as the first-line therapeutic option for pemphigus due to its ability to induce remission by depleting peripheral B lymphocytes.
View Article and Find Full Text PDFPemphigus is a rare autoimmune disease characterised by the production of pathogenic autoantibodies in response to different desmosome proteins. The pathophysiological process leads to the development of blisters and erosions on mucosal and/or skin surfaces. The classical clinical variants of pemphigus are pemphigus vulgaris and pemphigus foliaceus.
View Article and Find Full Text PDFBackground: Atopic dermatitis (AD) presents with the wide spectrum of clinical phenotypes within and between various populations. Recent study showed low frequency of filaggrin loss-of-function (FLG LOF) mutations in Croatian AD patients. At present, there are no data on biomarkers of immune response in Croatian AD patients that might be useful in the selection and monitoring of novel immune therapies.
View Article and Find Full Text PDFBackground: The incidence of skin cancers has been increasing steadily over the last decades. Although there have been significant breakthroughs in the management of skin cancers with the introduction of novel diagnostic tools and innovative therapies, skin cancer mortality, morbidity and costs heavily burden the society.
Objective: Members of the European Association of Dermato-Oncology, European Academy of Dermatology and Venereology, International Dermoscopy Society, European Dermatology Forum, European Board of Dermatovenereology of the European Union of Medical Specialists and EORTC Cutaneous Lymphoma Task Force have joined this effort to emphasize the fundamental role that the specialist in Dermatology-Venereology has in the diagnosis and management of different types of skin cancer.
J Eur Acad Dermatol Venereol
September 2020
Background: Pemphigus encompasses a group of life-threatening autoimmune bullous diseases characterized by blisters and erosions of the mucous membranes and skin. Before the era of immunosuppressive treatment, pemphigus was almost always fatal. Due to its rarity, only few randomized controlled therapeutic trials are available.
View Article and Find Full Text PDFLichen planus (LP) is a chronic inflammatory and immune-mediated disease that affects the skin, hair, nails and mucous membranes. Although there is a broad clinical spectrum of lichen planus manifestations, the skin and oral cavity remain the major sites of involvement. A group of European dermatologists with a long-standing interest and expertise in lichen planus has sought to define therapeutic guidelines for the management of patients with LP.
View Article and Find Full Text PDFActa Dermatovenerol Croat
July 2020
Hidradenitis suppurativa (HS) is a chronic skin disease affecting hair follicles in intertriginous areas, characterized by deep, recurrent, painful nodules and abscesses, fistulae, sinus tracts, and scarring. With a prevalence of 1-4%, HS is not an uncommon disease. Several risk factors have been linked with the development of HS, such as genetic predisposition, smoking, and obesity, leading to the hypothesis that HS develops as a result of environmental triggers in a genetically susceptible individual.
View Article and Find Full Text PDFAt the beginning of the 28th year of the life of our Acta Dermatovenerologica Croatica Journal, we experienced so many challenges which are quite difficult to grasp of and which nobody could predicted in advance. It all began with some vague information from Wuhan about a new virus which was spreading quickly and which eventually led to a lock-down in our country as well. We had hoped that this would not have such a big impact on the life of the Journal, in fact we hoped that may some of our potential authors would have more time to finalize their previous research and send us their manuscripts.
View Article and Find Full Text PDFAim: To evaluate the association between the use of dipeptidyl peptidase-4 inhibitors (DPP4I) and clinical and laboratory findings of bullous pemphigoid (BP) in patients treated at the European Reference Network - Skin Reference Centre in Croatia.
Methods: This retrospective study enrolled 82 patients treated for BP at the Department of Dermatovenereology, University Hospital Center Zagreb from January 2015 to December 2019. Clinical features of BP, presence of comorbidities, and laboratory findings of anti-BP antibodies and eosinophilia were analyzed in three groups of BP patients: 1) diabetes mellitus (DM) type II patients treated with DPP4I, 2) DM type II patients not treated with DPP4I, and 3) non-DM type II patients.