Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

suspected tracheolaryngeal stenosis, stricture, or obstruction

1st line – tracheostomy

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ACUTE
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suspected tracheolaryngeal stenosis, stricture, or obstruction
1st line – 

tracheostomy

Tracheolaryngeal involvement occurs in up to about half of all junctional EB (JEB), severe children, and a smaller percentage of those with other JEB subtypes, during early childhood.[43][65]

It may be sudden and, if not acted on promptly, fatal.

The risk of this complication plateaus by about 6 years of age.

Considered in any infant or small child with JEB who has evidence of upper airway involvement (e.g., a hoarse cry).[43] Demands immediate evaluation by an otolaryngologist.[62]

If the upper airway is even partially compromised, then many EB experts recommend tracheostomy to prevent the later occurrence of sudden airway obstruction and death.

Tracheostomy may be removed in later childhood or young adulthood without recurrence of clinically significant tracheolaryngeal disease activity.

suspected systemic infection

1st line – broad-spectrum antibiotics to cover likely pathogens or known sensitivity

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ACUTE
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suspected systemic infection
1st line – 

broad-spectrum antibiotics to cover likely pathogens or known sensitivity

Systemic infection (usually bacterial) is a complication seen usually only in neonates and infants and needs to be treated immediately. A broad-spectrum antibiotic should be given intravenously, based on the most likely organism(s) involved, with further alterations based on the results of cultures.

Sepsis primarily occurs in more severely affected children (severe EB simplex, severe junctional EB; and severe recessive dystrophic EB). This may occur as a result of widespread erosions on the skin and/or within mucosal surfaces.

May result in death.[66]

ONGOING

EBS, JEB, DEB, Kindler EB

1st line – dressings

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ONGOING
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EBS, JEB, DEB, Kindler EB
1st line – 

dressings

Patients with epidermolysis bullosa simplex (EBS), junctional EB (JEB), dystrophic EB (DEB), or Kindler EB should receive sterile or clean dressings to open wounds followed by tubular or rolled gauze bandaging for cushioning. Types of dressing include nonadherent/semi-adherent synthetic dressings, petroleum-impregnated gauze, and silver-impregnated dressings.

Gentle daily cleansing of the skin is recommended. Severe generalized wounds may benefit from occasional baths or soaks in diluted chlorine-containing solutions.

To prevent blisters forming, clean padded dressings or wraps are applied to those skin areas (e.g., elbows and knees) where the greatest amount of mechanical traction may occur.

A consensus report is available that summarizes the variety of dressings that may be of value in the treatment of EB.[51] Dressing choices should be individualized based on EB subtype, extent, and wound location, as well as dressing frequency, cost, and availability.

Plus – sterile drainage of blisters

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ONGOING
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EBS, JEB, DEB, Kindler EB
Plus – 

sterile drainage of blisters

Treatment recommended for ALL patients in selected patient group

Blisters require sterile drainage to enhance healing and reduce pain.

Consider – topical antibacterial agent

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ONGOING
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EBS, JEB, DEB, Kindler EB
Consider – 

topical antibacterial agent

Treatment recommended for SOME patients in selected patient group

Skin wounds re-epithelialize best if covered with an ointment to produce a moist and slightly anaerobic environment.

Applying a mild topical antibiotic ointment to the surface of any such wound, unless the patient is known to be allergic to ≥1 of its components, may be beneficial.

Polymyxin-containing products or bacitracin is the preferred choice.

Topical antiseptics may be an alternative choice.

Occasionally wounds are covered instead with silver-impregnated dressings.

Silver sulfadiazine may be used in those wounds that are superficially infected with gram-positive bacteria despite wound coverage with other agents.

Mupirocin ointment is used only on poorly responding wounds that are infected with culture-confirmed Staphylococcus aureus that do not require systemic antibiotic therapy. Chronic widespread use may predispose to overgrowth by MRSA.

Primary options

bacitracin/neomycin/polymyxin B topical: apply to the affected area(s) with each dressing change

OR

bacitracin topical: (5000 units/g) apply to the affected area(s) with each dressing change

Secondary options

silver sulfadiazine topical: (1%) apply to the affected area(s) with each dressing change

Tertiary options

mupirocin topical: (2%) apply to the affected area(s) with each dressing change

Plus – nutritional supplementation

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ONGOING
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EBS, JEB, DEB, Kindler EB
Plus – 

nutritional supplementation

Treatment recommended for ALL patients in selected patient group

Nutritional supplementation consists of protein and carbohydrate-enriched liquid supplements and multivitamins with zinc, selenium, carnitine, and iron.

Affected individuals benefit from formal dietetic reviews. Vitamin D insufficiency has been observed to be common particularly in children with recessive dystrophic EB (RDEB), necessitating supplementation.[52] Vitamin C deficiency has also been identified.[53]

Feeding gastrostomies may be useful in enhancing nutritional intake in severely affected children who are unable to take in sufficient amounts of nutrients by mouth (especially those with RDEB and JEB).[65]

Difficulties with sucking, due to the presence of painful blisters or erosions along the palate, may be managed using modified feeding nipples.

Attention should also be given to bowel habit, as affected individuals can often develop multifactorial constipation, in part due to diet, opioid analgesia, relative immobility and diminished fluid intake.[54]

Plus – lifestyle advice and monitoring for complications

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ONGOING
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EBS, JEB, DEB, Kindler EB
Plus – 

lifestyle advice and monitoring for complications

Treatment recommended for ALL patients in selected patient group

Although avoiding mechanical trauma is the basis of preventing blistering in EB, affected children and adults should be encouraged to pursue as full a lifestyle as is practical.

In milder forms of EB, some sporting activities may even be possible.

Formal physical therapy, occupational therapy and speech therapy input can significantly enhance daily capabilities.​​[57][58]

It is critical that children develop as psychologically normal as possible and specialist psychological support is frequently beneficial in supporting this.[59]

Home schooling is unnecessary for most EB children, and social interactions with peers are extremely important for the wellbeing of the child and the parents. However, many occupations involving strenuous physical activity, including military service, are not a possibility.

The day-to-day care of children and adults with EB is usually provided by pediatricians, dermatologists and internists. Any extracutaneous complication, including severe anemia, growth retardation, or involvement of any of the targeted extracutaneous organs, should have prompt medical or surgical intervention.

Consider – aluminum chloride

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ONGOING
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EBS, JEB, DEB, Kindler EB
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EBS
Consider – 

aluminum chloride

Treatment recommended for SOME patients in selected patient group

Topical aluminum chloride may be used as an astringent to the soles and/or palms to reduce blistering secondary to hyperhidrosis.[56]

Discontinued if excessive dryness develops.

Primary options

aluminum chloride topical: (20%) apply to the affected area(s) once daily as tolerated

Consider – tetracycline

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ONGOING
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EBS, JEB, DEB, Kindler EB
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EBS
Consider – 

tetracycline

Treatment recommended for SOME patients in selected patient group

Tetracyclines antibiotics (e.g., tetracycline) may be used as a possible suppressive agent to reduce blister formation.[55]​ Tetracycline antibiotics also have anti-inflammatory properties. Up to 4 months of therapy may be required, to determine potential efficacy.

They are generally used only in children >8 years of age, because earlier use may result in permanent discoloration of tooth enamel.

Primary options

tetracycline: 250-500 mg orally twice daily

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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