Ann Dermatol.  2016 Feb;28(1):6-14. 10.5021/ad.2016.28.1.6.

Quality of Life and Economic Burden in Recessive Dystrophic Epidermolysis Bullosa

• Jeon IK
• On HR
• Kim SC

• KMID: 2429476
• DOI: http://doi.org/10.5021/ad.2016.28.1.6

Abstract

BACKGROUND
Patients with recessive dystrophic epidermolysis bullosa (RDEB) exhibit blisters and erosions since birth, causing pain, pruritus and various complications. RDEB affects quality of life (QoL) in physical, emotional and social aspects. Furthermore, interminable dressing changes and supportive therapies impose a significant economic burden on the patient's family.
OBJECTIVE
We assessed the QoL and economic burden in patients with RDEB.
METHODS
Sixteen patients with RDEB were surveyed to assess the QoL and economic burden. Patients answered questionnaires consisting of a visual analogue scale (VAS) on pain and pruritus, Skindex-29, Quality of Life in EB questionnaire (QOLEB), and the economic burden due to EB.
RESULTS
Thirteen patients with RDEB completed the questionnaire. Female patients presented higher VAS, QOLEB and total Skindex-29 scores than male patients. Patients with RDEB showed severe levels of pruritus, which was more intolerable than pain. Mean VAS score on pain in RDEB was higher than in oral lichen planus and post-herpetic neuralgia. VAS score on pruritus was similar to those in chronic urticaria, atopic dermatitis, and prurigo nodularis. Compared with other dermatologic conditions, patients with RDEB were profoundly affected in all three scales of skindex-29. Mean "medical cost" in a month was $257.54 (USD) (+/-169.39) and mean "dressing cost" was $358.41 (USD) (+/-312.55), which was negatively related to patient age.
CONCLUSION
RDEB had a profound impact on QoL and economic burden. Compared with other dermatologic diseases, RDEB showed severe symptoms and QoL was seriously impaired. Most patients sustained economic burdens, especially on preparing dressing materials. Younger patients experienced more economic burdens.

Keyword

Dressing; Economic burden; Quality of life; Recessive dystrophic epidermolysis bullosa

MeSH Terms

Bandages
Blister
Dermatitis, Atopic
Epidermolysis Bullosa Dystrophica*
Female
Humans
Lichen Planus, Oral
Male
Neuralgia
Parturition
Prurigo
Pruritus
Quality of Life*
Urticaria
Weights and Measures

Figure

• 

  Fig. 1 Visual analogue scale (VAS) in (A) pain and (B) pruritus. VAS differences of 6~10 mm are considered as clinically meaningful. (A) The mean pain scale in recessive dystrophic epidermolysis bullosa (RDEB) is higher than in oral lichen planus and herpes zoster (post-herpetic neuralgia). (B) The mean pruritus scale is similar to that in chronic urticaria, atopic dermatitis, and prurigo nodularis.

• 

  Fig. 2 Questions and answers about economic burden and dressing materials. (A) Seven patients answer that they experienced economic burden on dressing materials "always", and 3 patients answered "often". (B) Seven patients change the dressing every day, and 4 patients change three times a week. (C) Seven patients answer that they require 1~2 hours to change their dressings, and 4 patients answered less than 1 hour. (D) Eleven patients (84.6%) answer that specialized dressings are better or much better in function than general dressings. (E) Price is the most important factor when purchasing dressings, followed by pain during dressing change. (F) Regarding fixing materials, 12 patients answer that the specialized fixing materials are better than general fixing materials. (G) Price and fixing ability are two important factors when selecting fixing materials.

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