Search results for "Scleroderma"

Nodular morphea keloidal type: A rare case with paradigmatic histopathology significantly accompanied by a flawless surgical scar

2020

Nodular morphea is a rare variant of localized scleroderma, clinically and histopathologically characterized by cutaneous nodules or plaques associated or superimposed to the flat lesions of classic morphea. Accordingly, the association of such outgrowths with systemic sclerosis is designated as nodular scleroderma. Sometimes these lesions appear as firm, erythematous and irregularly curvy plaques resembling keloids or hypertrophic scars, thus characterizing keloidal morphea or keloidal scleroderma. These mystifying features can make the diagnosis challenging, especially in the absence of a well‐documented medical history. Here we report a case of keloidal morphea with multiple histopatholo…

Morphea “en coup de sabre”: an unusual oral presentation

2017

Morphea, or localized scleroderma, is an inflammatory disease that leads to sclerosis of the skin and underlying tissues due to excessive collagen deposition. Oral involvement is unusual and it may produce white linear fibrotic areas with a scar-like appearance, atrophy of tongue papillae, gingival recession and alveolar bone resorption. We report a case of a 13-year-old girl who consulted for progressive recession on the attached gingiva of her upper left incisors. She also presented a hypopigmented line on the left side skin of her upper lip, which continued through the vermilion and the lip mucosa, including the gingiva of the affected teeth. Clinical examination, blood tests, computeriz…

Risk of scleroderma according to the type of immune checkpoint inhibitors

2020

Abstract Introduction Immune checkpoint inhibitors (ICIs) are associated with immune-related adverse events (irAEs). Among them, ICIs-induced systemic sclerosis (SSc) is poorly known. Methods To better characterize this irAE, our comprehensive approach combined the description of ICIs-induced scleroderma cases, the systematic review of the literature and the analysis of VigiBase, the WHO pharmacovigilance database. Results We identified two cases with underlying limited cutaneous SSc who presented a dramatic increase in the skin thickening following pembrolizumab, associated with scleroderma renal crisis in one case. In the literature, four cases of scleroderma and four cases of morphea hav…

Rehabilitative interventions for ischaemic digital ulcers, pain, and hand functioning in systemic sclerosis: a prospective before-after study

2022

Abstract Background Systemic sclerosis (SSc) is a rare connective tissue disease characterised by immune dysfunction, vasculopathy, cellular inflammation, fibrosis of the skin associated with multiple internal organs involvement. Ischaemic digital ulcers (IDU) of the hands commonly occur in patients with SSc adversely affecting functional independence. Purpose Aim of the study is to investigate the effectiveness of a rehabilitation protocol based on the combined use of ultrasound (US) therapy and therapeutic exercise in terms of ulcers healing, pain relief, and hand functioning in patients affected by SSc with IDUs. Moreover, we also investigated the safety of the proposed intervention. Stu…

Chapter 13 Antiphospholipid Antibodies and Vasculitis

2009

Abstract Besides the large group of primary vasculitis diseases, vasculitis in autoimmune diseases is most commonly associated with systemic lupus erythematosus (SLE), scleroderma, rheumatoid arthritis, insulin-dependent diabetes mellitus, and dermatomyositis. The antiphospholipid syndrome (APS), as a relatively recently recognized autoimmune disorder, may also present with a variety of vasculitis features. These non-typical vasculitis syndromes have to be considered very carefully in the APS, and vasculitis manifestations presenting as systemic involvement in APS patients have to be identified very early to prevent patients from rapidly developing organ damage. The aim of this review artic…

Endothelial progenitor cells: Are they displaying a function in autoimmune disorders?

2016

Endothelial Progenitor Cells (EPCs) are bone marrow derived cells able to differentiate in mature endothelial cells (EC) contributing to the generation of new vessels, connecting to fibronectin, and forming colonies and/or colony forming units. Since circulating EPCs can be actively considered part of endothelial damage in several cardiovascular diseases and autoimmune disorders the possibility to have a measure for endothelium damage should be considered of interest to predict the patient out-come. At the same time the EPCs proliferative and regenerative role could be considered for therapeutic applications. Studies have been performed to elucidate the role of EPCs in Systemic Sclerosis an…

Lipofilling and platelet rich plasma, Hyaluronic acid and platelet rich plasma: new perspectives for scleroderma patients

2016

Implants in patients with oral manifestations of autoimmune or muco-cutaneous diseases – A systematic review

2018

Background To give an overview on implant survival rates in patients with oral manifestations of systemic autoimmune (oral Lichen planus (oLp), Pemphigus (Pe)), muco-cutaneous (Epidermolysis bullosa (EB)), autoimmune multisystemic rheumatic diseases (Sjögren´s syndrome (SjS), systemic Lupus erythematosus (sLE), or systemic Sclerosis (sSc)). Material and Methods Systematic literature review (PubMed/Medline, Embase) using MESH and search term combinations, published between 1980 and August 2018 in English language reporting on dental implant-prosthetic rehabilitation of patients with oLp, Pe, EB, SjS, sLE, sSc, study design, age, gender, follow-up period (≥ 12 months), implant survival rate. …

COVID‐19 and exacerbation of dermatological diseases: A review of the available literature

2021

Abstract Since the emergence of the new coronavirus disease 19 (COVID‐19) pandemic, there has been a concern for the patients with chronic autoimmune diseases including dermatological conditions over the potential exacerbation of these underlying conditions after infection with severe acute respiratory syndrome coronavirus‐2 (SARS‐CoV2). We performed a systematic review to evaluate presentations, postinfection change in the manifestation, diagnosis, and management of flare‐ups of underlying dermatologic disease in patients with COVID‐19. A total of 17 articles were recovered reporting on flare‐ups of dermatological disease including pemphigus vulgaris, psoriasis, subacute cutaneous lupus er…

The skin in autoimmune diseases-Unmet needs.

2016

Treatment of skin manifestations in systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and dermatomyositis (DM) is based on the results of only few randomized controlled trials. The first-line treatment for disfiguring and widespread cutaneous involvement in SLE is antimalarials, but some patients are therapy resistant. Recently, the monoclonal antibody belimumab was approved for SLE as an adjunct therapy for patients with autoantibody-positive disease who despite standard therapy show high disease activity, intolerance of other treatments, or an unacceptably high need for corticosteroids. However, a validated skin score has not been used to confirm the efficacy of belimumab on m…