Epidermolysis bullosa (EB) has no cure and its treatment takes several factors into consideration, including areas affected, physiological response to healing, infection and involvement of other organ systems.
In addition to these, the four main types of EB - dystrophic epidermolysis bullosa (DEB), epidermolysis bullosa simplex (EBS), junctional epidermolysis bullosa (JEB), and Kindler syndrome (KS) - contribute to how well or poor the aforementioned factors play out.
Therapy involves a multidisciplinary approach and aims at preventing complications and easing the pain of the blisters.
The multidisciplinary team needed in the management of EB includes doctors, nurses, psychologists, and patient support groups.
Drugs such as corticosteroids and other anti-inflammatory agents that may be used topically or orally are useful in EB to help control the pain and itching.
Surgical therapy involves skin grafting in areas where the lesions are very deep, removal of cancerous lesions that might have developed, dilation of the esophagus in cases of stricture or narrowing and repair of any deformities that arise.
Future therapies may target genes and proteins and some are currently under investigation.
The healing of wounds associated with EB may be impaired by factors such as age, nutritional deficiencies, bacterial infection, foreign bodies, tissue anoxia, and medications such as glucocorticoids.
It is imperative to avoid and treat these factors where applicable in order to ensure optimal wound healing.
For instance, foreign bodies need to be removed and infections need to be treated with the appropriate antimicrobial therapy based on the bacteria identified.
Persons with the JEB subtype of the disease may have retarded healing due to defects in laminin 5, which is a protein responsible for adhesion and migration of skin cells.
EB-associated esophageal symptoms may be treated with drugs such as phenytoin or oral steroids to reduce the pain that occurs with swallowing.
If candidiasis arises, then anti-candidal agents may be helpful. Regular dental visits are required for EB patients as good oral hygiene is essential, especially in patients with DEB and JEB, who are more prone to developing dental caries. In these patients, in particular, alcohol-containing mouthwashes should be avoided, as these are very harsh.
Ocular manifestations of the disease may be managed with lubricants, antibiotic ointments for infections, and cycoplegic agents to reduce spasms of the ciliary muscle.
Patients with EB need to avoid trauma or friction to the skin in order to help minimize the frequency of blistering.
Some advice in order to accomplish this include avoiding walking long distances, wearing comfortable shoes with support and padding, refraining from contact sports, keeping cool in warm weather, and not using clothing that fits too tightly or rubs against the skin.
Infection prevention strategies such as strict wound care regimens in vulnerable areas of extensive crusting and denudation are crucial. Furthermore, patients must ensure they get adequate nutrition and physical therapy.
Educating the patient and his or her family members is important in order to maximize the quality of life and minimize the potential complications associated with the disease.
Reviewed by Susha Cheriyedath, MSc