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Dermatologic diseases and pregnancy
Published in Hung N. Winn, Frank A. Chervenak, Roberto Romero, Clinical Maternal-Fetal Medicine Online, 2021
Holly Edmonds, Dana Ward, Ann G. Martin, Susana Leal-Khouri
The sera of patients with pemphigoid gestationis contain an antibody to a basement membrane constituent of normal skin. This antibody, known as “herpes gestationis factor,” avidly binds complement and is an IgG1 molecule (47). Using conventional indirect immunofluorescence, only 25% of patients show evidence of “herpes gestationis factor,” and usually in low titers. However, complement added indirect immunofluorescence reveals the circulating anti-basement membrane zone IgG in nearly all patients, thus this technique is much more sensitive (38). Salt split skin studies reveal the immunoreactants to localize in the epidermal side of the lamina lucida, a finding similar to that seen in bullous pemphigoid (48). Placental studies reveal a similar deposition of C3 and immunoglobulin along the amniotic basement membrane. As in lesional skin and plasma, IgG1 is the most frequently found immunoglobulin (49).
Dermoepidermal junction
Published in Lionel Fry, Atlas of Bullous Diseases, 2020
Since the antigens are components of the basement membrane region, it is important to know its structure. On electron microscopy the basement membrane, which separates the epidermis and dermis, is composed of an upper translucent area, the lamina lucida, and an electron-dense region, the lamina densa (Figure 4.1). The basal layer of ker-atinocytes is attached to the basement membrane by electron-dense structures termed hemidesmosomes. The structures have an intracellular and an extracellular component. The latter consists of anchoring filaments which traverse the lamina lucida and end in the lamina densa, whose constituents include laminin 5 and type IV collagen. Below the lamina densa are anchoring fibrils composed of type VII collagen which anchor the lamina densa to the dermis.
Dermatology
Published in Rachel U Sidwell, Mike A Thomson, Concise Paediatrics, 2020
Rachel U Sidwell, Mike A Thomson
A more severe form involving blisters within the lamina lucida of the epidermis. All areas of skin affected, nails are shed. Internal blistering of the respiratory and gastrointestinal tracts, mid-facial erosions, hoarse voice, stridor.
Granzyme B as a therapeutic target: an update in 2022
Published in Expert Opinion on Therapeutic Targets, 2022
Alexandre Aubert, Michael Lane, Karen Jung, David J. Granville
Pemphigoid diseases (PD) are a group of seven autoimmune disorders characterized by subepithelial blistering in response to autoantibody production against structural components of the dermal-epidermal junction (DEJ) [57]. The DEJ is a multiprotein complex that comprises architectural hemidesmosomal proteins of the BM (Laminins and ColIV), ColVII anchoring fibrils, as well as cell surface receptors (ColXVII and α6β4 integrins). Altogether, hemidesmosomes attach the intracellular cytoskeleton of basal keratinocytes to the lamina lucida and lamina densa of the BM, thus delineating the stratum basale of the epidermis and maintaining homeostasis. Hemidesmosomes also contribute to skin integrity by linking the epidermis/BM complex to the underlying dermal connective tissue (Figure 2, left panel) [58,59].
Current developments in gene therapy for epidermolysis bullosa
Published in Expert Opinion on Biological Therapy, 2022
Thomas Kocher, Igor Petkovic, Johannes Bischof, Ulrich Koller
In recent years, gene therapies for genodermatoses have steadily become the focus of research. Several therapeutic strategies, based on RNA/DNA repair or substitution, are already at both preclinical and clinical stage (Figure 1). The monogenetic skin disease epidermolysis bullosa (EB) represents a strong gene therapy target, as pathogenic mutations in at least 16 unique genes, which are crucial for skin integrity, result in severe phenotypes [1]. EB is characterized by the formation of extended blisters and lesions on the skin and mucous membranes upon minimal mechanical trauma. Clinical and genetic aspects, genotype–phenotype correlations as well as disease-modifying factors of EB were thoroughly reviewed by Has and colleagues [1]. With ~500,000 people affected worldwide, EB is a rare and very heterogeneous skin disease, which can be divided into four major subtypes. Mutations within keratin 5, 14 and plectin lead to epidermolysis bullosa simplex (EBS), associated with intraepidermal blistering, whereas junctional EB (JEB) is caused by mutations in genes coding for laminin-332, type XVII collagen (C17) and integrin-α6β4. This form of EB is characterized by blistering within the lamina lucida of the basement membrane. Mutations within COL7A1, encoding type VII collagen (C7), are responsible for a particularly severe form of EB, dystrophic EB (DEB), while Kindler syndrome is caused by mutations within the KIND1 gene [1,2].
Impairment of wound healing by reactive skin decontamination lotion (RSDL®) in a Göttingen minipig® model
Published in Cutaneous and Ocular Toxicology, 2020
Jessica M. Connolly, Robert S. Stevenson, Roy F. Railer, Offie E. Clark, Kimberly A. Whitten, Robyn B. Lee-Stubbs, Dana R. Anderson
Antibodies included (1) pan-laminin (Sigma L9393, dilution 1:100), (2) fibronectin (Sigma F6140, dilution 1:100), (3) collagen type VII (Sigma C6805, dilution 1:100) and (4) laminin 332 (Millipore MAB19562, dilution 1:40). Pan-laminin localises the lamina lucida of the basement membrane zone and appears consistently across the basement membrane zone when migration of the basal epithelium is almost completed and the cells are stationary. Fibronectin is a provisional matrix for wound healing that is expressed in the wound bed during early wound healing as a wide amorphous, dispersed material. It is tightly localised in the upper papillary dermis during basal epithelial cell migration (5–10 days). During later phases of wound-healing expression, it is more dispersed, amorphous and less intensely expressed. Collagen type VII, which demonstrates anchoring fibrils, is used to indicate dermal/epidermal adhesion and appears as a late event in wound healing. Laminin 332 is located in the lamina lucida and associated with hemidesmosomes. Disruption of laminin 332 localisation can represent poor basal epithelial cell adhesion.