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Related Concept Videos

Type IV Collagen of Basal Lamina01:05

Type IV Collagen of Basal Lamina

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Type IV collagen is a 400 nm long, network-forming collagen that acts as a barrier between the epithelial and endothelial cells. Type IV collagen  forms the backbone of the basement membrane by scaffolding with laminin, entactin, proteoglycans, and fibronectin. Apart from rendering structural support to the basement membrane, it also helps entail signaling potentials necessary for both pathological and physiological functions.
A type IV collagen molecule has six alpha chains which can...
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Fibril-associated Collagen01:11

Fibril-associated Collagen

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Fibril-associated collagens are a type of collagens present in the extracellular matrix with interrupted triple helices or FACIT (Fibril-associated collagens interrupted triple-helices). FACIT help connect and attach the collagen fibrils with each other as well as with other proteins of the extracellular matrix.
For example, the type II collagen fibrils in cartilage have covalently bound type IX fibril-associated collagens at regular intervals. Other types of fibril-associated collagens are...
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Introduction to Connective Tissues01:11

Introduction to Connective Tissues

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Connective tissues are one of the four main tissue types in humans that are extensively present in the body. They are characterized by cells embedded in an extracellular matrix (ECM) composed of a ground substance and three main types of protein fibers— collagen, elastic, and reticular fibers. The ground substance of connective tissues can range from a watery and jelly-like consistency to mineralized and hard. The wide variety of cells in the connective tissues include fibroblasts,...
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Structural Protein Function01:56

Structural Protein Function

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Structural proteins are a category of proteins responsible for functions ranging from cell shape and movement to providing support to major structures such as bones, cartilage, hair, and muscles. This group includes proteins such as collagen, actin, myosin, and keratin.
Collagen, the most abundant protein in mammals, is found throughout the body. In connective tissue, such as skin, ligaments, and tendons, it provides tensile strength and elasticity.  In bones and teeth, it mineralizes to...
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Collagens are the Major Structural Proteins of ECM01:13

Collagens are the Major Structural Proteins of ECM

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Three main types of fibers are secreted by fibroblasts: collagen fibers, elastic fibers, and reticular fibers. Collagen fiber is made from fibrous protein subunits linked together to form a long, straight fiber. Collagen fibers, while flexible, have great tensile strength, resist stretching, and give ligaments and tendons their characteristic resilience and strength. These fibers hold connective tissues together, even during the body's movement.
Connective tissue proper includes loose...
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Connective Tissue Cell Types01:22

Connective Tissue Cell Types

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Connective tissue develops from the mesoderm of a developing embryo and consists of cells, fibers, and ground substance: a gel-like material containing large complexes of carbohydrates and proteins. Connective tissue was first identified as a separate tissue family in the 18th century, and Johannes Peter Muller coined the term connective tissue.
Fat cells (adipocytes), smooth muscle cells (myoblasts), and bone cells (osteoblasts) are some connective tissue cell types. Some immune system cells...
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Related Experiment Video

Updated: Jan 15, 2026

Tissue Engineering of Tumor Stromal Microenvironment with Application to Cancer Cell Invasion
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Type VII Collagen Disorders Simplified.

Charles Camisa1

  • 1OnSpot Dermatology, Naples, Florida, and Riverchase Dermatology, Fort Myers, Florida.

Cutis
|October 7, 2025
PubMed
Summary

Epidermolysis bullosa acquisita (EBA) and bullous systemic lupus erythematosus (BSLE) are autoimmune diseases targeting type VII collagen. Recent advances offer new treatments for dystrophic epidermolysis bullosa (DEB) and junctional epidermolysis bullosa (JEB).

Area of Science:

  • Dermatology
  • Genetics
  • Immunology

Background:

  • Epidermolysis bullosa acquisita (EBA) and bullous systemic lupus erythematosus (BSLE) are autoimmune diseases targeting type VII collagen.
  • Type VII collagen is crucial for skin and mucous membrane integrity, forming anchoring fibrils that connect epithelium to connective tissue.
  • Dystrophic epidermolysis bullosa (DEB) shares clinical features with EBA and BSLE but results from COL7A1 gene mutations.

Purpose of the Study:

  • To differentiate between EBA, BSLE, and DEB.
  • To review current and emerging treatment strategies for these conditions.

Main Methods:

  • Diagnosis involves personal/family history, histopathology, immunopathology, autoantibody profiling, electron microscopy, and gene mutation analysis.
  • Treatment for EBA and BSLE includes immunosuppressants and antineutrophil drugs, with rituximab showing success in resistant cases.

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  • DEB and other heritable epidermolysis bullosa (EB) treatments were historically supportive but now include newly approved advances.

    • Main Results:

    • Differentiating these rare conditions requires comprehensive diagnostic approaches.
    • Established treatments for EBA and BSLE have limitations, but rituximab offers an alternative.
    • Significant therapeutic progress has been made for DEB and junctional EB.

    Conclusions:

    • Accurate diagnosis of EBA, BSLE, and DEB is essential for appropriate management.
    • While EBA and BSLE treatments are evolving, new therapies are now available for DEB and JEB.
    • Advances in understanding and treating epidermolysis bullosa offer hope for patients with these rare skin conditions.