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Dupilumab: drug re-purposing for dystrophic epidermolysis bullosa

Dr Liat Samuelov and Prof Eli Sprecher from the Division of Dermatology, Tel-Aviv Medical Center, Israel, kindly provide information about the option to use the drug Dupilumab for the treatment of itch in EB based on their experiences and reported cases.

Portrait of woman with black hair on the left and a man with grey hair on the right

Dr. Liat Samuelov & Prof. Eli Sprecher; Division of Dermatology, Tel-Aviv Medical Center

Dupilumab is the first biologic therapy approved for the treatment of moderate to severe atopic dermatitis (AD), a common inflammatory skin disease characterized by pruritic erythematous lesions frequently associated with other allergic conditions and elevated immunoglobulin E (IgE) levels. Dupilumab is a fully humanized monoclonal antibody targeting the interleukin (IL)-4 receptor alpha (IL-4Rα), which is a receptor subunit shared by two type 2 cytokines IL-4 and IL-13, which play an important role in the pathogenesis of AD.

Apart from being an effective treatment for AD, dupilumab has been shown to be effective for the treatment of generalized pruritus and prurigo nodularis (extremely pruritic lesions over the extremities) suggesting that it may possibly benefit patients without AD and affected by conditions associated with severe itch such as EB.
Itch in EB is not only associated with markedly decreased quality of life and daily functioning, it also triggers the development of new mechanical trauma-induced lesions.

We and others recently reported the successful treatment of EB-associated intractable itch with dupilumab. The first reported case was a 52-year-old female diagnosed with dystrophic EB pruriginosa (EBP) associated with scarring lesions over her extremities and persistent local itch interfering with all daily functions. She had failed countless topical and systemic anti-pruritic therapies. Dupilumab treatment was initiated and following 4 and 12 weeks of treatment, significant improvement in pruritus, quality of life and EB disease severity was observed (Shehadeh et al, Br J Dermatol, 2020). No side effects were recorded.

The beneficial effects of dupilumab in dystrophic EB have since been confirmed in several reports (Zhou et al, Pediatr Dermatol, 2021; Clawson et al, JAAD Case Rep, 2021; Wang et al, Dermatol Therap, 2021; Caroppo et al, J Eur Acad Dermatol Venereol, 2021).
Severe pruritus in EB has been attributed to aberrant activation of inflammatory circuits. It has recently been shown that many EBP patients demonstrate elevated IgE levels with no evidence of atopy but with Th2 skewing, strongly supporting a role for Th2-mediated immunity in this condition and providing a mechanistic explanation for the therapeutic effect of dupilumab.

In conclusion, dupilumab is a potentially effective and safe treatment for EB patients suffering from disabling itch.

For questions about the experiences and about the article please get in touch directly with Dr Samuelov.

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