Epidermolysis bullosa

It must always be subjected to medical evaluation, and it is very important to keep in mind the adverse effects and the possible pharmacological interactions. 

Topical

• Although not all treatments have the same evidence regarding its effectivity, some of the usually recommended ones for direct application on the skin are:
  • Topical corticosteroids, especially when the itching is very severe and of acute onset. It is especially important in this case to follow the medical guidelines regarding the frequency and duration of treatment.
  • Topical antihistamine drugs should not be used.
  • Topical anaesthetics, although rare due to their adverse effects, should also be avoided.
  • Other drugs that have been used are: calcineurin inhibitor drugs.

Systemic

They are treatments that have effects at a general level of the entire body, especially drugs that affect the nervous system. Although EB-specific evidence is lacking, there are many treatments extrapolated from patients with large burns or from patients with neuropathic pain. Here is a summary of the available treatments:

• Antihistamines. Usually used for allergies, these drugs have a relative effect in EB since the itching in people with epidermolysis has a different origin than in allergy. However, they are mainly used for their sedative effect at night, helping to fall asleep and to minimize scratching that occurs during the night.
• Antidepressants (tricyclics and/or selective serotonin reuptake inhibitors). These drugs are commonly used for the management of depressive mood states, which in some cases are present in patients with chronic itching. In addition, they have a sedative side effect that helps manage itching.
• HT3 receptor antagonists. These drugs are usually recommended for the management of nausea, such as ondansetron or granisetron, but they have the side effect of controlling itching. Its use should be reserved for cases of very complicated itching.
• Anticonvulsantsand antipsychotic agents. Its use should be reserved for cases of very complicated and difficult to manage itching.
• Opioid antagonists, such as naloxone, which can counteract the pruritic effect of drugs such as morphine. However, they must be used very carefully because they can lead to an increase of the pain.

There are other treatments which have been reported as anecdotal or experimental:cyproheptadine, serlopitant (NK1 receptor antagonist); monoclonal antibodies (dupilumab or TNF inhibitors); cyclosporine, thalidomide, dapsone, cannabinoids, or apremilast. When the itch is related to an infection, the treatment with antibiotics might help the itchiness as it would directly be treating the cause.

As a conclusion, itchiness is a prevalent symptom in EB and of difficult treatment, in which only a drug or intervention is usually not enough. For this reason, interventions on the environment (temperature, humidity, clothing, ...), psychological therapies and drugs must be combined for greater control of this annoying symptom.