Topical Diacerein Ointment for Epidermolysis Bullosa Simplex

A Review

Allison L. Limmer, BA, BS; Crystal E. Nwannunu, BS; Radhika Shah, BS, MS; Kendall Coleman, BSA; Ravi R. Patel, MD; Uyen Ngoc Mui, MD; Stephen K. Tyring, MD, PhD

Disclosures

Skin Therapy Letter. 2019;24(3):7-9. 

In This Article

Pathogenesis of Epidermolysis Bullosa

EB often results from either an autosomal dominant (AD) or autosomal recessive (AR) genetic defect.[6] Mutations of genes encoding integumentary proteins are the most common causes of poor integrity and skin fragility that lead to blistering in patients with EB.[5] EB has been categorized into 4 major types, the most common of which is epidermolysis bullosa simplex (EBS) with an incidence of about 1 in every 25,000–50,000 people.[7] EBS is usually inherited in an AD pattern. Defects frequently occur in the keratin filament genes, KRT5 and KRT14, and these mutations lead to destabilization of keratinocyte cytoskeletons within the epidermis. EBS presents with blisters affecting the hands and feet, normally resolving without signs of scarring.[5]

The major risk factor for this disease is a family history of EB. Prevalence may be found in every racial and ethnic group worldwide and affects both sexes equally.[2]

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