A group of diseases that may receive less attention in the international medical field are called non-communicable diseases. These diseases are caused by a variety of causes in the person, some congenital at birth and some during one’s life due to various causes such as lifestyle or environmental complications. Epidermolysis bullosa (EB) is one of those diseases, A non-communicable disease (NCD) that is rather overlooked in medical fields all around the world.
There are different statistics regarding EB prevalence. According to some resources, it affects one out of 50,000 live births. A number of other resources state that EB affects 20 in every one million live births; however, considering the overall world population and Infant mortality rate, 9 out of one million people around the world suffer from EB.
Of the total affected population, 92% suffer from epidermolysis bullosa simplex (EBS), which is the most common type of EB, and 8% struggle with dystrophic epidermolysis bullosa (DEB) and junctional epidermolysis bullosa (JEB) which are more severe.
The prevalence rates of EB disease carriers – people who are not affected but inherited the defective gene from one of their parents – are reported to be one out of 450 people for DEB and one out of 333 people for JEB. The prevalence rate of EBS carriers is estimated to be much higher than the other two types.
Gender differences or various racial and ethnic groups do not influence EB occurrence. Therefore, the distribution of EB is relatively equal among the world population, including Iran. Nevertheless, there are a number of factors which can slightly influence the distribution of EB in Iran. These factors are briefly described below.
One risk factor that can increase the rate of EB development in babies is the marriage of two EB carriers. Although chances are normally low, this risk factor inflicts societies with high rates of inter-family marriage since defective genes are likely to be present in most members of a given family.
Iran is one of the countries with high rates of intermarriage. According to the studies by the Iranian EB Home conducted in Iran:
- More than 90% of the people affected with EB are born to intermarried parents.
- The patient count is higher in provinces with more traditional customs and beliefs.
- The patient count in a family is higher when the family’s intermarriages are more intertwined.
Based on world statistics, EB patient count in Iran with a population of over 88 million people should be approximately between 1,000 to 1,500. However, due to the high rate of inter-family marriages in Iran, EB patient count is probably above the global average.
EB Home, the official provider of EB patients in Iran, has identified a significant percentage of EB patients so far. The overall statistics are as follows:
- A total of 820 patients have been identified since 2016. Of all patients, more than 650 people have official records and regularly receive a variety of healthcare, psychiatric, and basic social services. Unfortunately, since the establishment of EB Home, 53 of these patients have died due to lack of familiarity with the disease, inadequate medical coverage, and sometimes level of disease progression. Although there’s no data for mortality rate prior to the establishment of EB Home.
- The province of Tehran has the highest EB patient count due to several reasons such as centrality, different racial and ethnic groups, and high population density. The southern provinces of Iran like Khuzestan, Sistan and Baluchistan, and Fars also have the highest EB patient counts after Tehran.
- The presented statistics were prepared up to August 2019. The goal of EB Home is to identify all EB patients in Iran, and the Foundation will continue pursuing this goal until it is fully achieved.
In order to facilitate patient identification and registration, EB Home has created a user-friendly patient registration portal to identify and cover new patients who are not yet supported by EB Home. Data of the newly registered patients are reviewed and confirmed by EB specialist physicians after patients’ online registration and document completion. Then, our social work unit makes contact with each of the patients in order to identify and take necessary measures to fulfill their needs. In addition to the facilitation of patient identification and registration, this approach has a significant positive impact on providing more effective and comprehensive services to EB patients.
-EB Home Public Relations