Evaluation of Treatments for Pruritus in Epidermolysis Bullosa

Abstract

Background Pruritus is a common complication in patients with epidermolysis bullosa (EB) and can be problematic. Objective data about the treatments used by EB patients for pruritus have not been reported and recommendations are limited. Objective To quantitatively determine which treatments have been used by EB patients for pruritus and to evaluate the perceived effectiveness of these treatments in pruritus relief. Methods A questionnaire was developed to evaluate which treatments and therapies have been used for pruritus in patients of all ages and types of EB. Questions about bathing products, moisturizers, topical products, oral medications, dressings, and alternative therapies were included. A 5-point Likert scale (-2=relieves itch a lot, -1=relieves itch a little, 0=no change, 1=increases itch a little, 2=increases itch a lot) was used to evaluate the perceived effectiveness of different treatments on pruritus relief. Patients from seven North American EB centers were invited to participate. Results Greasy ointments (53.4%), lotions (45.2%), creams (40.4%), and oral hydroxyzine (39.0%) were the most frequently used treatments for pruritus. Treatments that were used frequently and perceived to be the most effective included creams (mean=-1.1), topical prescription corticosteroids (mean=-1.0), oils (mean=-0.9), oral hydroxyzine (mean=-0.9), topical diphenhydramine (mean=-0.9), and vaporizing rub (menthol/camphor/eucalyptus) (mean=-0.9). Patients that used creams (p=0.05) or lotions (p=0.04) more often experienced significantly less pruritus. Systemic opioids (mean=0.3), adherent bandages (mean=0.3), and bleach baths (mean=0.2) slightly increased pruritus. Conclusions Randomized-controlled trials of therapies will be necessary to develop evidence-based recommendations for control of pruritus in EB patients.

Reliability and validity of the instrument for scoring clinical outcomes of research for epidermolysis bullosa (iscorEB)

“…For instance, EBQOL includes one question about pain, whereas iscorEB‐p assesses overall pain in addition to skin, mouth, eye and bone/joint pain. The iscorEB‐p also assesses itch, which has been found to be a significant concern of patients with EB . Unlike EBQOL, iscorEB‐p does not emphasize independence, relationships, finances and emotional complexities (other than mood).…”

“…The iscorEB-p also assesses itch, which has been found to be a significant concern of patients with EB. 20 Unlike EBQOL, iscorEB-p does not emphasize independence, relationships, finances and emotional complexities (other than mood). We do not view these differences as a deficiency of iscorEB-p. Rather, they simply highlight our emphasis on assessing outcomes that may change as a result of intervention and outcomes that are perceived to be important by patients affected by EB.…”

Restoration of cutaneous pigmentation by transplantation to mice of isogeneic human melanocytes in dermal–epidermal engineered skin substitutes

“…Engineering of skin substitutes for tissue repair and regenerative medicine presents numerous challenges to development of confidence in reproducibility of clinical outcomes. The data reported here address the specific limitations to reproducible restoration of skin color after grafting for skin loss conditions, including burns, and congenital skin lesions such as giant nevus and epidermolysis bullosa (EB; Danial et al., ; Passaretti et al., ; Pfendner & Lucky, ).…”

Beneficial Effect of Systemic Allogeneic Adipose Derived Mesenchymal Cells on the Clinical, Inflammatory and Immunologic Status of a Patient With Recessive Dystrophic Epidermolysis Bullosa: A Case Report

“…As a result, patients exhibit different grades of malnutrition, anemia and inflammation (40-42) via complex interrelationships not fully understood. Peripheral neuropathology associated with inflammation (43, 44) may be underlying the chronic itch and pain, which are the first and second highest disease burdens for patients living with RDEB, respectively (45)(46)(47).…”