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Parry Romberg Syndrome (PRS) and en coup de sabre (ECDS) are head variants of linear morphea with functional and structural implications. This study describes the clinical course, autoimmune co-morbidities, complications, and treatment of adults with PRS/ECDS at a tertiary referral center. We retrospectively reviewed the records of all 34 adult patients with PRS/ECDS identified through billing code search and seen by dermatologists at our institution between 2015 and 2021. Eight patients (23.5%) had ECDS, 8 (23.5%) had PRS, and 18 (52.9%) had overlap. Twenty-six patients (76.5%) reported ocular, oral, and/or neurologic symptoms, and 8 (23.5%) had concomitant autoimmune/inflammatory conditions. Sixteen patients (47.1%) had a skin biopsy, and 25 (73.5%) had imaging. Forty-six MRIs were obtained, of which 6 (13.0%) reported intracranial findings and 25 (54.3%) reported disease-related connective tissue damage. Twenty-four patients (70.6%) underwent systemic treatment during their disease course per available clinical records. Seventeen patients (70.8%) had improved or stable disease upon treatment completion, with an average duration of 22.2 months. Ten patients (41.7%) reported recurrence of disease following the treatment course. To address changes to facial contour, 6 patients (17.6%) opted for procedural treatments. One patient (16.7%) experienced morphea reactivation following a filler injection performed off-immunosuppression. Compared to findings in children, our study suggests adults with PRS/ECDS are more likely to have oral and ocular complications but experience less severe neurologic symptoms. While systemic treatments appear beneficial in most adult patients with PRS/ECDS, disease may recur following discontinuation.

While mucocutaneous manifestations of COVID-19 have been frequently reported and added to our knowledge every day during the pandemic, another issue is the COVID-related diseases that can present as intensified lesions of underlying diseases, a new disease, or changes in the behavior of an old lesion. Given that immune system overreaction and cytokine storm are among the most prominent events in COVID-19, the incidence of autoimmune diseases is expected to increase after COVID-19, as confirmed in several reports. To increase the body of knowledge about short- and long-term outcomes of COVID-19 for specialists, it is essential that similar cases be reported and collected for years to come. The present study investigated a case of pansclerotic morphea that rapidly progressed a few weeks after infection with COVID-19 in a 57-year-old woman with no history of any autoimmune skin or rheumatic diseases. She was prescribed outpatient COVID-19 treatment of azithromycin, vitamins D and C, and then quarantined for 2 weeks. The manifestations of the disease were exacerbated at each follow-up and sampling visit at short intervals. This kind of pansclerotic morphea is reported for the first time.

Abstract Rationale Analyzing the molecular heterogeneity of different forms of organ fibrosis may reveal common and specific factors and thus identify potential future therapeutic targets. Objectives We sought to use proteome-wide profiling of human tissue fibrosis to (1) identify common and specific signatures across end-stage interstitial lung disease (ILD) cases, (2) characterize ILD subgroups in an unbiased fashion, and (3) identify common and specific features of lung and skin fibrosis. Methods We collected samples of ILD tissue (n = 45) and healthy donor control samples (n = 10), as well as fibrotic skin lesions from localized scleroderma and uninvolved skin (n = 6). Samples were profiled by quantitative label-free mass spectrometry, Western blotting, or confocal imaging. Measurements and Main Results We determined the abundance of more than 7,900 proteins and stratified these proteins according to their detergent solubility profiles. Common protein regulations across all ILD cases, as well as distinct ILD subsets, were observed. Proteomic comparison of lung and skin fibrosis identified a common upregulation of marginal zone B- and B1-cell–specific protein (MZB1), the expression of which identified MZB1+/CD38+/CD138+/CD27+/CD45−/CD20− plasma B cells in fibrotic lung and skin tissue. MZB1 levels correlated positively with tissue IgG and negatively with diffusing capacity of the lung for carbon monoxide. Conclusions Despite the presumably high molecular and cellular heterogeneity of ILD, common protein regulations are observed, even across organ boundaries. The surprisingly high prevalence of MZB1-positive plasma B cells in tissue fibrosis warrants future investigations regarding the causative role of antibody-mediated autoimmunity in idiopathic cases of organ fibrosis, such as idiopathic pulmonary fibrosis.

Morphea, also known as localized scleroderma, is a chronic inflammatory connective tissue disorder with variable clinical presentations, that affects both adults and children. It is characterized by inflammation and fibrosis of the skin and underlying soft tissue, in certain cases even of the surrounding structures such as fascia, muscle, bone and central nervous system. While the etiology is still unknown, many factors may contribute to disease development, including genetic predisposition, vascular dysregulation, T H 1/T H 2 imbalance with chemokines and cytokines associated with interferon-γ and profibrotic pathways as well as certain environmental factors. Since the disease may progress to permanent cosmetic and functional sequelae, it is crucial to properly assess the disease activity and to initiate promptly the adequate treatment, thus preventing subsequent damage. The mainstay of treatment is based on corticosteroids and methotrexate. These, however, are limited by their toxicity, especially if applied long-term. Furthermore, corticosteroids and methotrexate often do not sufficiently control the disease and/or the frequent relapses of morphea. This review presents the current understanding of morphea by discussing its epidemiology, diagnosis, management and prognosis. In addition, it will describe recent pathogenetic findings, thus proposing potential novel targets for therapeutic development in morphea.

[This corrects the article DOI: 10.3389/fimmu.2024.1351675.].

Linear morphea (LM) is a chronic autoimmune disease characterized by linear or band-like localized sclerosis that may involve the dermis, subcutaneous tissue, muscle, and underlying bone. While LM typically presents in childhood, adult-onset disease is considerably less common, and LM presenting during breastfeeding has not, to our knowledge, been previously documented. As a result, therapeutic guidance for managing LM in breastfeeding women remains extremely limited, highlighting the need for additional clinical evidence in this population. A 44-year-old breastfeeding woman presented with brownish-white skin lesions with a line configuration and hardened skin on the right shoulder, upper arm, forearm, hand, and fingers. The histopathological findings demonstrated epidermal thinning with flattened rete ridges, thickened fibrocollagenous connective tissue stroma extending into the subcutaneous fat, hyaline degeneration, a mild lymphocytic inflammatory, and absence of pilosebaceous unit. The laboratory analysis revealed a positive ANA test with a titer of 1:320 and the presence of anti-RNP and anti-Sm autoantibodies. The patient received hydroxychloroquine (HCQ) 200 mg twice daily and topical pimecrolimus 1% cream twice daily. After three months of observation, there was an improvement in the skin lesions. In addition, the modified localized skin index (mLoSSI) score and the localized scleroderma damage index (LoSDI) score decreased from 6 to 1 and 5 to 4, respectively. No adverse effects related to retinal toxicity were reported during the course of therapy. This case suggests that the combination of oral HCQ and topical pimecrolimus may represent a safe and effective therapeutic option for LM in breastfeeding women, although further evidence is needed to confirm long-term outcomes.

Skin autoimmune conditions belong to a larger group of connective tissue diseases and primarily affect the skin, but might also involve underlying tissues, such as fat tissue, muscle, and bone. Autoimmune antibodies (autoantibodies) play a role in autoimmune skin diseases, such as localized scleroderma also termed morphea, and systemic scleroderma, also called systemic sclerosis (SSc). The detailed studies on the biological role of autoantibodies in autoimmune skin diseases are limited. This results in a few available tools for effective diagnosis and management of autoimmune skin diseases. This review aims to provide an update on the detection and most recent research on autoantibodies in morphea. Several recent studies have indicated the association of autoantibody profiles with disease subtypes, damage extent, and relapse potential, opening up exciting new possibilities for personalized disease management. We discuss the role of existing autoantibody tests in morphea management and the most recent studies on morphea pathogenesis. We also provide an update on novel autoantibody biomarkers for the diagnosis and study of morphea.

We report typical cases of biopsy confirmed granuloma annulare occurring on irradiated skin in the context of breast cancer in three women in their seventies. The lesions appeared immediately after start of irradiation in one patient, 4 and 15 months after the end of radiation treatment in the other two. The lesions eventually spread elsewhere on the body in two patients. Similar to morphea, which is a well‐known side effect of irradiation, we believe that radiotherapy can induce tissue damage, leading to dermal inflammation ultimately responsible for the emergence of granuloma annulare.