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Journal of Lower Genital Tract Disease Jul 2021The aim of the study was to identify whether erosive lichen sclerosus (LS) is a distinct clinicopathologic subtype.
OBJECTIVE
The aim of the study was to identify whether erosive lichen sclerosus (LS) is a distinct clinicopathologic subtype.
MATERIALS AND METHODS
The pathology database was searched for "erosion," "erosive," "ulcer," and "lichen sclerosus." Inclusion criteria were histopathologic diagnosis of LS and erosion or ulcer overlying a band of hyalinization and/or fibrosis. Exclusions were concurrent neoplasia and insufficient tissue. Histopathologic review documented site, epithelial thickness, adjacent epidermal characteristics, infiltrate, and dermal collagen abnormality. Clinical data included demographics, comorbidities, examination findings, microbiologic results, treatment, and response.
RESULTS
Ten examples of erosive LS and 15 of ulcerated LS occurred in 24 women with a mean age of 67 years. Ulcerated LS was associated with diabetes and nontreatment at time of biopsy. Clinicians identified red patches in all but 1 case of erosive LS. Ulcerated LS was documented as fissure, ulcer, or white plaque, with 8 (53%) described as lichenified LS with epidermal breaches. Erosive LS favored hairless skin with normal adjacent stratum corneum sloping gently into erosion, whereas most ulcers in LS had an abrupt slope from hair-bearing skin. All cases were treated with topical steroids; 2 patients with erosive LS and 10 with ulcerated LS also had oral antifungals, topical estrogen, antibiotics, and/or lesional excision. Treatment yielded complete resolution in 50%.
CONCLUSIONS
Erosive LS is an unusual clinicopathologic subtype characterized by red patches on hairless skin seen microscopically as eroded epithelium overlying a band of hyalinized or fibrotic collagen. In contrast, ulcerated LS is usually a traumatic secondary effect in an uncontrolled dermatosis.
Topics: Aged; Female; Humans; Lichen Sclerosus et Atrophicus; Middle Aged; Vulvar Lichen Sclerosus
PubMed: 33859124
DOI: 10.1097/LGT.0000000000000607 -
Acta Dermatovenerologica Alpina,... Dec 2023Autoimmune blistering skin diseases (AIBDs) encompass several heterogeneous conditions clinically characterized by blisters and erosions on the skin and mucous membranes... (Review)
Review
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. The diagnostic methods for AIBDs have made tremendous progress in the last 2 decades due to the availability of standardized serological assays that allow precise diagnosis in most patients. If left untreated, these diseases are potentially life-threatening due to superinfections and loss of body fluids, and in some severe cases due to restricted food intake. Based on the available literature, this paper provides an overview of the clinical and immunopathological characteristics of the most common AIBDs.
Topics: Humans; Autoantibodies; Autoimmune Diseases; Blister; Pemphigoid, Bullous; Pemphigus
PubMed: 38126096
DOI: No ID Found -
Italian Journal of Dermatology and... Jun 2023The human skin barrier is structurally and functionally immature at birth, with elevated skin surface pH, lower lipid content, and lower resistance to chemicals and...
The human skin barrier is structurally and functionally immature at birth, with elevated skin surface pH, lower lipid content, and lower resistance to chemicals and pathogens. Infants at risk for atopic dermatitis (AD) may present with xerosis almost immediately after birth. The current algorithm on skincare for newborns and infants aims to promote a healthy skin barrier and potential mitigation of AD. The project used a modified Delphi hybrid process comprising face-to-face discussions followed by an online follow-up replacing a questionnaire. During the meeting, a panel of eight clinicians who treat newborns and infants discussed the systematic literature review results and a draft algorithm addressing non-prescription skincare for neonates and infants. Online the panel reviewed and adopted the algorithm using evidence coupled with the panel's expert opinion and clinical experience. The algorithm provides clinical information for pediatric dermatologists, dermatologists, and pediatric healthcare providers treating neonates and infants. The advisors adopted a scale based on clinical signs for the algorithm: 1) scaling/xerosis; 2) erythema; and 3) erosion/oozing. Skincare for newborns and infants includes: aim for a cool environment and soft cotton clothing, give lukewarm baths (~5 min, 2-3 x week) with consideration of a gentle cleanser (pH 4-6) and the application of a full-body moisturizing after bath, while avoiding products with toxic and irritating ingredients. A growing body of evidence recognizes the benefits of ongoing daily use of non-alkaline cleansers and moisturizers. Gentle cleansers and moisturizers containing barrier lipids help maintain the protective skin barrier when applied from birth onwards.
Topics: Humans; Infant; Infant, Newborn; Child; Dermatitis, Atopic; Skin; Skin Care; Erythema; Health Status; Autonomic Nervous System Diseases
PubMed: 37278500
DOI: 10.23736/S2784-8671.23.07336-X -
Clinical Chemistry and Laboratory... 2006Bullous skin diseases represent a group of organ-specific autoimmune disorders characterised by binding of circulating autoantibodies to adhesion molecules of the... (Review)
Review
Bullous skin diseases represent a group of organ-specific autoimmune disorders characterised by binding of circulating autoantibodies to adhesion molecules of the epidermis and the dermo-epidermal basement membrane zone. Binding of these autoantibodies to their antigenic targets results in loss of adhesion between epidermal keratinocytes and at the level of the basement membrane zone. Chronic blisters and secondary painful erosions are the clinical hallmark of autoimmune bullous disorders. Histopathology reveals the location of blister formation and helps to classify the subtype of the bullous skin disorder. Immunofluorescence is crucial for diagnosing autoimmune bullous skin disorders. Tissue-bound autoantibodies are detected by direct immunofluorescence of perilesional skin. Circulating autoantibodies can be visualised by indirect immunofluorescence using tissue substrates such as monkey oesophagus and sodium chloride-split human skin. Most of the autoantigens are available as recombinant proteins, which allows for autoantibody screening by ELISA or immunoblot analysis to confirm the primary diagnosis and, importantly, for immunoserological follow-up of patients.
Topics: Autoantibodies; Autoimmune Diseases; Diagnosis, Differential; Fluorescent Antibody Technique; Humans; Skin Diseases, Vesiculobullous
PubMed: 16475898
DOI: 10.1515/CCLM.2006.027 -
Life (Basel, Switzerland) Dec 2022Erosive pustular dermatosis (EPD) is a chronic inflammatory skin disorder that usually affects mature individuals. It predominantly affects the scalp and can lead to... (Review)
Review
Erosive pustular dermatosis (EPD) is a chronic inflammatory skin disorder that usually affects mature individuals. It predominantly affects the scalp and can lead to scarring alopecia. Risk factors include actinic damage and androgenetic alopecia. A traumatic insult to the skin is considered a vital trigger of the condition. EPD is a diagnosis of exclusion; thus, several neoplastic, infectious, vesiculobullous, and inflammatory conditions should be ruled out. Biopsy and clinicopathologic correlation are required to differentiate between EPD and these entities. A dysregulated, chronic immune response is considered central to the etiopathogenesis of EPD. We performed an evidence-based systematic review of the management options. There were predominantly studies with level IV and V evidence and only two with level III. Despite the responsiveness of EPD to potent topical steroids, such as clobetasol propionate, recurrence occurs after treatment withdrawal. With the available data, tacrolimus 0.1%, curettage-assisted aminolevulinic acid-photodynamic therapy, and systemic retinoids can be considered second-line options for EPD with a role in maintenance regimens. However, controlled data and more powerful studies are needed to make solid recommendations.
PubMed: 36556462
DOI: 10.3390/life12122097 -
Journal of Wound, Ostomy, and... 2019Stomal and peristomal skin complications (PSCs) are prevalent in persons living with an ostomy; more than 80% of individuals with an ostomy will experience a stomal or...
Stomal and peristomal skin complications (PSCs) are prevalent in persons living with an ostomy; more than 80% of individuals with an ostomy will experience a stomal or peristomal complication within 2 years of ostomy surgery. Peristomal skin problems are especially prevalent, and a growing body of evidence indicates that they are associated with clinically relevant impairments in physical function, multiple components of health-related quality of life, and higher costs. Several mechanisms are strongly linked to PSCs including medical adhesive-related skin injuries (MARSIs). Peristomal MARSIs are defined as erythema, epidermal stripping or skin tears, erosion, bulla, or vesicle observed after removal of an adhesive ostomy pouching system. A working group of 3 clinicians with knowledge of peristomal skin health completed a scoping review that revealed a significant paucity of evidence regarding the epidemiology and management of peristomal MARSIs. As a result, an international panel of experts in ostomy care and peristomal MARSIs was convened that used a formal process to generate consensus-based statements providing guidance concerning the assessment, prevention, and treatment of peristomal MARSIs. This article summarizes the results of the scoping review and the 21 consensus-based statements used to guide assessment, prevention, and treatment of peristomal MARSIs, along with recommendations for research priorities.
Topics: Adhesives; Consensus; Humans; Prevalence; Skin; Skin Care; Surgical Stomas
PubMed: 30844869
DOI: 10.1097/WON.0000000000000513 -
International Journal of Molecular... Dec 2016Toxic epidermal necrolysis (TEN) is a rare but life threatening mucocutaneous reaction to drugs or their metabolites. It is characterised by widespread keratinocyte... (Review)
Review
Toxic epidermal necrolysis (TEN) is a rare but life threatening mucocutaneous reaction to drugs or their metabolites. It is characterised by widespread keratinocyte apoptosis and sloughing of the skin, erosions of the mucous membranes, painful blistering, and severe systemic disturbance. The pathophysiology of TEN is incompletely understood. Historically, it has been regarded as a drug-induced immune reaction initiated by cytotoxic lymphocytes via a human leukocyte antigen (HLA)-restricted pathway. Several mediators have been identified as contributors to the cell death seen in TEN, including; granulysin, soluble Fas ligand, perforin/granzyme, tumour necrosis factor-α (TNF-α), and TNF-related apoptosis-inducing ligand. Currently, granulysin is accepted as the most important mediator of T cell proliferation. There is uncertainty around the accepted management of TEN. The lack of definitive management guidelines for TEN is explained in part by the rarity of the disease and its high mortality rate, which makes it difficult to conduct randomised control trials on emerging therapies. Developments have been made in pharmacogenomics, with numerous HLA alleles identified; however, these have largely been ethnically specific. These associations have translated into screening recommendations for Han Chinese.
Topics: Antigens, Differentiation, T-Lymphocyte; Apoptosis; Cyclosporine; Drug-Related Side Effects and Adverse Reactions; Epidermis; Fas Ligand Protein; Granulocyte Colony-Stimulating Factor; Granzymes; Humans; Immunoglobulins, Intravenous; Infliximab; Keratinocytes; Mucous Membrane; Perforin; Stevens-Johnson Syndrome; TNF-Related Apoptosis-Inducing Ligand; Tumor Necrosis Factor-alpha
PubMed: 27999358
DOI: 10.3390/ijms17122135 -
F1000Research 2018Skin fragility refers to a large group of conditions in which the ability of the skin to provide protection against trivial mechanical trauma is diminished, resulting in... (Review)
Review
Skin fragility refers to a large group of conditions in which the ability of the skin to provide protection against trivial mechanical trauma is diminished, resulting in the formation of blisters, erosions, wounds, or scars. Acquired and physiological skin fragility is common; genetic disorders are rare but give insight into the molecular mechanisms ensuring skin stability. The paradigm is represented by inherited epidermolysis bullosa. This review is focused on recent advances in understanding the molecular basis of genetic skin fragility, including emerging concepts, controversies, unanswered questions, and opinions of the author. In spite of the advanced knowledge on the genetic causes of skin fragility, the molecular pathology is still expanding. Open questions in understanding the molecular basis of genetic skin fragility are the following: what are the causes of phenotypes which remain genetically unsolved, and what are the molecular modifiers which might explain phenotypic differences among individuals with similar mutations? New mutational mechanisms and new genes have recently been discovered and are briefly described here. Comprehensive next-generation sequencing-based genetic testing improved mutation detection and facilitated the identification of the genetic basis of unclear and new phenotypes. Characterization of the biochemical and cell biological consequences of the genetic variants is challenging and laborious but may represent the basis for personalized therapeutic approaches. Molecular modifiers of skin fragility have been uncovered in particular animal and genetic models but not in larger cohorts of patients. This scientific progress is the basis for revisions of the epidermolysis bullosa classification and for innovative therapeutic approaches designed for this intractable condition.
PubMed: 29568501
DOI: 10.12688/f1000research.12658.1 -
American Journal of Medical Genetics.... Oct 2014Ankyloblepharon-ectodermal defects-cleft lip/palate (AEC) syndrome is a rare monogenetic disorder that is characterized by severe abnormalities in ectoderm-derived... (Review)
Review
Ankyloblepharon-ectodermal defects-cleft lip/palate (AEC) syndrome is a rare monogenetic disorder that is characterized by severe abnormalities in ectoderm-derived tissues, such as skin and its appendages. A major cause of morbidity among affected infants is severe and chronic skin erosions. Currently, supportive care is the only available treatment option for AEC patients. Mutations in TP63, a gene that encodes key regulators of epidermal development, are the genetic cause of AEC. However, it is currently not clear how mutations in TP63 lead to the various defects seen in the patients' skin. In this review, we will discuss current knowledge of the AEC disease mechanism obtained by studying patient tissue and genetically engineered mouse models designed to mimic aspects of the disorder. We will then focus on new approaches to model AEC, including the use of patient cells and stem cell technology to replicate the disease in a human tissue culture model. The latter approach will advance our understanding of the disease and will allow for the development of new in vitro systems to identify drugs for the treatment of skin erosions in AEC patients. Further, the use of stem cell technology, in particular induced pluripotent stem cells (iPSC), will enable researchers to develop new therapeutic approaches to treat the disease using the patient's own cells (autologous keratinocyte transplantation) after correction of the disease-causing mutations.
Topics: Animals; Cleft Lip; Cleft Palate; Disease Models, Animal; Ectodermal Dysplasia; Epidermis; Eye Abnormalities; Eyelids; Humans; Induced Pluripotent Stem Cells; Mutation; Tumor Suppressor Proteins
PubMed: 24665072
DOI: 10.1002/ajmg.a.36455