Practical considerations relevant to treatment with the gene therapy beremagene geperpavec-svdt for dystrophic epidermolysis bullosa.

Background/purpose: Dystrophic epidermolysis bullosa (DEB), a rare genetic skin disease caused by loss-of-function mutations in , the gene encoding type VII collagen (COL7), is characterized by skin blistering, scarring, and extracutaneous manifestations that markedly reduce patient quality-of-life. Beremagene geperpavec-svdt ('B-VEC') is a gene therapy employing a non-integrating, replication-defective herpes simplex virus type 1 (HSV-1)-based vector encoding two copies of full-length human to restore COL7 protein after topical administration to DEB wounds. B-VEC was approved in the United States in 2023 as the first topical gene therapy and the first approved treatment for DEB.

In vivo topical gene therapy for recessive dystrophic epidermolysis bullosa: a phase 1 and 2 trial.

Recessive dystrophic epidermolysis bullosa (RDEB) is a lifelong genodermatosis associated with blistering, wounding, and scarring caused by mutations in COL7A1, the gene encoding the anchoring fibril component, collagen VII (C7). Here, we evaluated beremagene geperpavec (B-VEC), an engineered, non-replicating COL7A1 containing herpes simplex virus type 1 (HSV-1) vector, to treat RDEB skin. B-VEC restored C7 expression in RDEB keratinocytes, fibroblasts, RDEB mice and human RDEB xenografts.

In vivo examination of healthy human skin after short-time treatment with moisturizers using confocal Raman spectroscopy and optical coherence tomography: Preliminary observations.

Skin is our barrier against environmental damage. Moisturizers are widely used to increase hydration and barrier integrity of the skin; however, there are contrasting observations on their in vivo effects in real-life settings. In cosmetic studies, corneometers and tewameters are traditionally used to assess skin hydration.

Simultaneous immunofluorescence and histology in pemphigus vulgaris using ex vivo confocal laser scanning microscopy.

Ex vivo confocal laser scanning microscopy (ex vivo CLSM) provides rapid, high-resolution imaging and immunofluorescence examinations of the excised tissues. We aimed to evaluate the applicability of ex vivo CLSM in histomorphological and direct immunofluorescence (DIF) examination of pemphigus vulgaris (PV). 20 PV sections were stained with fluorescent-labeled anti-IgG and anti-C3 using various dilutions and incubation periods.

New-generation diagnostics in inflammatory skin diseases: Immunofluorescence and histopathological assessment using ex vivo confocal laser scanning microscopy in cutaneous lupus erythematosus.

Ex vivo confocal laser scanning microscopy (CLSM) offers real-time examination of excised tissue in reflectance, fluorescence and digital haematoxylin-eosin (H&E)-like staining modes enabling application of fluorescent-labelled antibodies. We aimed to assess the diagnostic performance of ex vivo CLSM in identifying histopathological features and lupus band test in cutaneous lupus erythematosus (CLE) with comparison to conventional histopathology and direct immunofluorescence (DIF). A total of 72 sections of 18 CLE patients were stained with acridine orange (AO), anti-IgG, anti-IgM and anti-IgA; 21 control samples were stained with AO.

Immunofluorescence and histopathological assessment using ex vivo confocal laser scanning microscopy in lichen planus.

Ex vivo confocal laser scanning microscopy (CLSM) provides rapid, high-resolution imaging, fluorescence detection and digital haematoxylin-eosin (H&E)-like staining. We aimed to assess the performance of ex vivo CLSM in identifying histomorphology and immunoreactivity in lichen planus (LP) and comparing its accuracy with conventional histopathology and direct immunofluorescence (DIF). Thirty-three sections of 17 LP patients stained with acridine orange (AO) and FITC-labelled anti-fibrinogen antibody and 21 control samples stained with AO were examined using ex vivo CLSM.

Recurrence of Pemphigus Vulgaris Under Nivolumab Therapy.

For many types of cancer, immune checkpoint inhibitors have proven to be a highly effective treatment. The monoclonal anti-PD-1 antibody nivolumab stimulates the immune system and is one of the newest treatment options for non-small cell lung cancer. In doing so, immune checkpoint inhibitors can trigger many skin lesions that have not yet been completely investigated in their entirety.

Effects of Short-Term Moisturizer Application in Different Ethnic Skin Types: Noninvasive Assessment with Optical Coherence Tomography and Reflectance Confocal Microscopy.

Background/aims: Ethnic skin types are known to differ in their morphological and physiological features. Thus, treatment responses may vary among different races. We aimed to assess skin morphology of different ethnicities and to compare the effect of short-term moisturizer application using optical coherence tomography (OCT) and reflectance confocal microscopy (RCM).

IL-31: A new key player in dermatology and beyond.

IL-31 is a novel cytokine expressed in many human tissues and involved mainly in T2-weighted inflammation. IL-31 signals through a receptor complex consisting of IL-31 receptor α and oncostatin M receptor β. The available data show that IL-31 is strongly linked with chronic pruritic skin disorders, such as atopic eczema, and represents a novel target for directed drug therapy.

Reflectance confocal microscopic evaluation of nonmelanocytic lip lesions.

Lips display various benign and malignant lesions. Considering their functional and cosmetic importance, noninvasive diagnostic methods are required. In vivo reflectance confocal microscopy (RCM) has already been reported to be useful in the evaluation of various skin lesions.

Bullous pemphigoid.

Bullous pemphigoid (BP) is the most common autoimmune bullous disorder which is characterized by autoantibodies against hemidesmosomal proteins of the skin and mucous membranes. Collagen XVII and dystonin-e have been identified as target antigens. BP affects mostly the elderly.

Diagnostic Value of Linear Fluorescence Along the Basement Membrane of Sweat Gland Ducts in Bullous Pemphigoid.

Linear IgG deposits along the basement membrane of adnexa has been proposed to be useful in the diagnosis of bullous pemphigoid (BP), but no controlled studies have been performed. This study evaluated linear IgG fluorescence of the basement membrane of sweat gland ducts (SGD) and other adnexa in perilesional biopsies from patients with BP (n = 64) and controls (n = 82), using direct immunofluorescence microscopy. Fluorescence intensity was graded semi-quantitatively.

The reflectance confocal microscopy in diagnosis of recurrent basal cell carcinoma.

Background: Reflectance confocal microscopy (RCM) is very valuable, non-invasive diagnostic tool in the diagnosis of basal cell carcinoma (BCC). Objective: We aimed to demonstrate the positive predictive value (PPV), negative predictive value, sensitivity and specificity of RCM and dermoscopic features in recurrent BCC.

Methods: The 152 entire lesion sites in 128 patients with BCC were evaluated by clinical examination, dermoscopy and RCM imaging techniques.

Successful treatment of generalized discoid lupus erythematosus with imiquimod cream 5%: a case report and review of the literature.

Discoid lupus erythematosus (DLE) is the most common form of chronic cutaneous lupus erythematosus. It tends to heal with scarring, hair loss, and pigmentary changes if treatment is not initiated in the early phase of the disease. Classic DLE lesions are initially red-purple macules, papules, or small plaques that rapidly acquire a hyperkeratotic appearance.