The rare skin disease epidermolysis bullosa (EB) is characterized by unusual blistering of the skin. Children with EB are called “butterfly children” because the skin is as delicate and fragile as a butterfly wing. Only about two to three out of 100,000 people are affected by the disease.
The cause is hereditary, the genes that determine the structure of protein molecules are affected, which in turn ensure that the different skin layers hold together. Epidermolysis bullosa is divided into the following forms:
- Epidermolysis bullosa simplex
- Dystrophic epidermolysis bullosa
- Junctional epidermolysis bullosa
- Kindler-syndrome
The difference between the individual forms lies in the respective gene alteration and the protein molecules affected. In addition to the aforementioned forms, there are about 40 other subtypes that differ in terms of the manifestation of the disease and the prognosis. What all patients have in common is that they have particularly sensitive skin.
Normally the first symptoms such as blisters, open skin and wounds appear already after birth.
There is currently no causal therapy or cure for the disease. Therefore, the focus is on pain therapy, wound care of the affected skin areas and the prevention of wounds. If contractures or adhesions occur, these can be treated with the help of a surgical intervention.
The endocannabinoid system and the skin
Studies have already shown that the skin contains endocannabinoid signalling pathways that are important for various processes. For example, researchers found out that the levels of the endocannabinoids anandamide and 2-AG in the skin increase sharply after an irritating stimulus (1). This is probably an adaptive response of the body to reduce inflammation and/or pain.
In the skin cells themselves, the endocannabinoid system also seems to play an important role. Both anandamide and 2-AG can influence the development of skin cells. This is especially true for the outermost cell layer of the skin (epidermis) as cannabinoid receptors have also been detected here (2).
Treatment with CBD oil
The researchers from Stanford University (USA) presented the results of their observational study on three cases in 2018 (3):
- Case 1: A six-month-old baby with severe EB was treated with an over-the-counter petrolatum ointment (Vaseline) and emu oil for wound care. In addition, the baby received the topical antibiotic mupirocin, the antihistamine diphenhydramine and morphine. During the study, a CBD spray was regularly applied to the affected skin areas.
- Case 2: A three-year-old girl with severe EB was treated with coconut oil, petrolatum ointment, zinc oxide allantoin 6 per cent cream and the topical antibiotic bacitracin. Subsequently, a mixture of CBD oil and emu oil was regularly applied to the sore areas.
- Case 3: A ten-year-old boy with severe EB was given an antibiotic, the painkiller naproxen and the anti-epileptic gabapentin, which is also used for nerve pain. CBD oil was then regularly applied to the affected skin areas.
In the first case, the blistering decreased, and the wounds healed faster. In addition, the baby needed less morphine. If we talk about the second and third cases, fewer blisters formed, and the wounds healed faster. There was also significant pain relief. The ten-year-old boy was even able to stop taking naproxen and gabapentin.
Treatment with THC and CBD
In 2019 researchers from the University of Groningen (The Netherlands) stated that opioid-based therapy can be used as a supportive treatment for epidermolysis bullosa. However, in a significant proportion of patients it could not provide sufficient pain relief. As cannabinoid-based medications are increasingly being investigated in the treatment of chronic pain, they represent a new option in the treatment of pain in EB.
The researchers presented three patients with EB who were prescribed pharmaceutical-grade sublingual cannabinoid-based medications consisting of tetrahydrocannabinol and cannabidiol. All three patients reported significant pain relief, reduced itching and dose reduction of general pain medication.
Sources:
(1) Bíró T, Tóth BI, Haskó G, Paus R, Pacher P. The endocannabinoid system of the skin in health and disease: novel perspectives and therapeutic opportunities. Trends Pharmacol Sci. 2009;30(8):411-420. doi:10.1016/j.tips.2009.05.004
(2) Maccarrone M, Di Rienzo M, Battista N, Gasperi V, Guerrieri P, Rossi A, Finazzi-Agrò A. The endocannabinoid system in human keratinocytes. Evidence that anandamide inhibits epidermal differentiation through CB1 receptor-dependent inhibition of protein kinase C, activation protein-1, and transglutaminase. J Biol Chem. 2003 Sep 5;278(36):33896-903. doi: 10.1074/jbc.M303994200. Epub 2003 Jun 18. PMID: 12815050.
(3) Chelliah MP, Zinn Z, Khuu P, Teng JMC. Self-initiated use of topical cannabidiol oil for epidermolysis bullosa. Pediatr Dermatol. 2018 Jul;35(4):e224-e227. doi: 10.1111/pde.13545. Epub 2018 May 22. PMID: 29786144.
(4) Schräder NHB, Duipmans JC, Molenbuur B, Wolff AP, Jonkman MF. Combined tetrahydrocannabinol and cannabidiol to treat pain in epidermolysis bullosa: a report of three cases. Br J Dermatol. 2019 Apr;180(4):922-924. doi: 10.1111/bjd.17341. Epub 2018 Nov 14. PMID: 30347109; PMCID: PMC7379189.