Table 9 Recommendations on EB patient’s management for pediatricians

Nutritional care

Soft diet

Oral intake 150–200%

Vitamin supplementation, iron supplementation

Gastrostomy, if necessary

6-12 months growth check

Gastroenterological care

Laxatives

Endoscopist evaluation

Esophageal dilatation

Dental care

Delicate oral hygiene

Avoiding sucrose

Dental prevention with programmed check

Emotional care

Psychological support for both patient and families/caregivers

Pain monitoring and treatment (NSAIDS and acetaminophen, tramadol, opioids, gabapentin)

Antihistamines (with eventually antidepressants and oral gabapentin or pregabalin) for chronic pruritus

Topical anesthetic for oral pain

Ocular care

Lubricants

Topical antibiotics in case of corneal erosions

Orthoptic surveillance for refractive errors and strabismus

Regular check

Mobility care

Physical specialists for mobility support

Footwear specific for EB patients

Plastic surgery in case of pseudo syndactyly in case of compromission of patients’ independence

Occupational therapy

Skin care

Advanced dressings, emollients, frictions avoidance

Skin infections treatment: bleach baths or compresses, topical antiseptics, and topical antibiotics. Bacterial cultures of critically colonized wounds are not routinely performed. If required, cultures should be obtained before starting topical antimicrobial treatment

Topical antiseptic agents include chlorhexidine, benzalkonium chloride, and silver sulfadiazine (small areas for short period because of side effects connected to systemic silver toxicity)

Topical antibiotics (mupirocin, fusidic acid) should be used with caution to avoid the antibiotic-resistant bacteria. If used, a rotation of different agents is recommended every two to six weeks to minimize the induction of bacterial resistance

In case of severe wound infections, systemic antibiotics based on antibiogram results

Regular assessment of skin lesions and multiple biopsies of chronic wounds in order to promptly diagnose skin cancer