Epidermolysis Bullosa in the Eastern Province of Saudi Arabia

Abstract

This study confirmed the rarity of epidermolysis bullosa in this province (population 3,000,000), and demonstrated the usefulness of oral phenytoin therapy, meticulous nursing, and good nutrition in the management of epidermolysis bullosa dystrophica.

The clinical spectrum of epidermolysis bullosa simplex

“…Comparison with other EB databases suggests that this population‐based study is equally if not more rigorous than other national surveys. 1 , 22–27 We have tried to minimize any bias towards more severely affected patients by actively seeking out EBS sufferers, approximately one‐third of whom proved to be previously unknown to dermatologists. 1 It is inevitable that some patients will remain undetected.…”

The clinical spectrum of dystrophic epidermolysis bullosa

“…Those affected by severe variants of EB are well known to the medical profession and are readily included in EB registers but, unless they are actively sought, sufferers with mild disease may not come to the attention of researchers. Assessments of the epidemiology of EB in other countries 2–9 suggest incomplete sampling, pronounced regional variation or differing genetic susceptibilities (Table 1). Although the ambitious U.S. national EB registry has so far published valuable information on the largest EB cohort, 9,10 the large size of the American population hinders complete sampling, thus resulting in a tendency to assess only more severely affected EB sufferers.…”

Dental and Anaesthetic Challenges in a Patient with Dystrophic Epidermolysis Bullosa

“…7 In Saudi Arabia, Abahussein et al reported the prevalence of EBS, JEB and DEB to be 1.7%, 0% and 3.7% per million, respectively; moreover, parental consanguinity was noted among 87.5% of cases. 8 There are no published studies on the exact prevalence of EB in Oman; however, unpublished data from national dermatology units indicate approximately 60 registered cases. This report focuses on the dental and anaesthetic challenges encountered during the management of an Omani adult patient with severe DEB.…”