Epidermolysis Bullosa (EB)
Epidermolysis bullosa (EB) is a group of rare genetic skin disorders in which the skin blisters with the slightest of trauma – a disease for which there is no cure. Depending on the type of E.B., these blisters occur anywhere on the body but are most common on the palms of the hands and soles of the feet, trunk, scalp, legs, and - in severe cases - inside the mouth, pharynx, esophagus. These blisters produce scarring which can be constricting.
Types of Epidermolysis Bullosa (EB)
The three most common types of E.B. are epidermolysis bullosa simplex (EBS), junctional epidermolysis bullosa (JEB) and dystrophic epidermolysis bullosa (DEB). EBS is the most common type (approximately 500,000 reported cases) typically autosomal dominant inheritance, and usually involves the palms and soles because of the trauma from daily activities like walking, playing ball, etc. Simple sitting and the rubbing of thighs while walking can create blisters on the buttocks or inner thighs. Heat and sweating can exacerbate the blister formation. The signs and symptoms usually occur in infancy but in mild cases may not show up until adolescence. EBS commonly creates reactions such as calluses over the palms and soles and thickness of nails. There is minimal mouth, throat or esophagus involvement.
A more severe type of EBS starts in infancy and can generate hundreds of blisters daily. This variety of EBS affects all aspects of life including a delay in walking. Although blistering starts at birth, it decreases with age.
Junctional Epidermolysis Bullosa (JEB)
The most severe, debilitating type of EB is junctional epidermolysis bullosa (JEB). This type is inherited in an autosomal recessive manner and is rare (approximately 7,000 patients globally). It commonly involves not only most body surfaces but also the mucous membranes. Because the blistering is at the basement membrane zone (a deeper layer than EBS), healing is slower, scarring is common, and deformity occurs including loss of hair follicles, fingernails and toenails. Teeth often have poorly formed enamel that causes discoloration, weakness and decay. The mouth, throat and esophagus are commonly involved which not only creates discomfort but also scarring and narrowing of the esophagus. This combination of symptoms usually leads to poor nutrition. Blistering can also be found in the urethra and can cause strictures. The resulting scar tissue is more prone to skin cancer formation and thus these patients must be monitored closely.
Although rare, JEB has a generalized severe type that, as the name suggests, is horrible and involves the entire epithelium of the body including the gastrointestinal tract and air way passages. Sadly, these patients rarely survive infancy.
Dystrophic Epidermolysis Bullosa (DEB)
Dystrophic epidermolysis bullosa (DEB) is genetically dominant or recessive. The dominant form has wide-spread clinical signs and symptoms that range from extensive blistering over much of the exposed skin and mouth to minimal blistering and only thickened nails. The recessive form of DEB is the most severe and generalized. Because of the severity, the scarring from this extensive blistering typically causes the fingers and toes to adhere together (mitten hands). This debilitating disease also affects the mouth, esophagus and arms. DEB patients also have a high skin cancer rate.
Treatment Needs for Epidermolysis Bullosa (EB)
Treatment should be based on preserving the viability of the blistered skin, protecting the surrounding skin from any trauma including adhesive irritation and dressing changes that are not traumatic to both the wound and surrounding skin. Wound change procedures should provide for longer dressing change intervals while being able to monitor signs of infection, as well as being able to clean blood and exudate from the wound as needed (up to two times a day) without disturbing the blistered skin.
Our Better Treatment Alternative for Epidermolysis Bullosa (EB)
Unfortunately, no such dressing existed until the inventions, research, development, patenting and manufacture of two transformative technologies: Miracle Dressing™ and Natural Marine Extract™ (NME™). The patent-pending Miracle Dressing is a 21-28 day stay-in-place dressing through which a health care provider can clean and medicate the wound without changing the dressing. Miracle Dressing creates a protective “second skin” barrier to prevent shear or friction forces and other traumas. This allows the blistered skin to heal without disruption while the surrounding skin remains trauma free. Miracle Dressing falls off on its own after 21-28 days which is usually when the blistered wound is healed. If needed, the Miracle Dressing Wound Care System can be re-applied. Lubrication is an important part of the healing process. We recommend the patented Natural Marine Extract™ in aloe vera which is included in the kit. It is composed of a revolutionary group of compounds, the ingredients of which have been shown to improve the speed and quality of damaged or wounded skin. (Please see our Natural Marine Extract ingredient references.) Sealing the micro-fissures of dry, atrophic skin improves the integrity, strength and resilience of the injured skin. Foam, silicone and standard adhesive barriers do not remove exudate or blood and do nothing to improve the quality of the underlying skin.
Please see the testimonial on our Burns page because the MDS treatment of burns is similar to the MDS treatment of EB wounds.