Epidermolysis Bullosa Activity and Scarring index (EBDASI)

Epidermolysis Bullosa Activity and Scarring index (EBDASI) is a scoring system that objectively quantifies the severity of Epidermolysis Bullosa (EB). The EBDASI is an essential tool for clinicians and patients to monitor the severity of the disease. It has also been designed to evaluate the response to new therapies for the treatment of EB.

Epidermolysis Bullosa

EB is a rare genetic skin disease, which is characterized by the formation of blisters and development of erosions and scarring after trauma to the skin. This genetic skin disease is classified into

• Epidermolysis Bullosa Simplex (EBS)
• Junctional Epidermolysis Bullosa (JEB)
• Dystrophic Epidermolysis Bullosa (DEB) .
• Kindlers syndrome

The classification above is based on mutations in genes that codes for the amino acids sequence of specific proteins in the skin that hold the skin together. New mutations can arise 'De Novo' in patients or it can be inherited either in a dominant or a recessive fashion, with the recessive types GeneRally being the most severe due to the inheritance of two affected copies of the gene. The severity of each type can vary even within a particular family.

Recent Advancement

In recent times the medical world has made great strides in the diagnosis and understanding of EB. Current treatment pertains to protective and therapeutic dressings to wounds, treatment of infections, use of topical preparations and prevention and treatment of complications of the disease including skin cancers, osteoporosis, renal failure and nutritional deficiencies.

The Basis Of The Scoring System

When studying disorders of the skin it is important to be able to measure the severity of a particular skin disease. A reproducible, recordable severity measure is essential to be able to monitor ProgresS or regression of the disease and to evaluate potential new therapeutic modalities. Most skin diseases, EB included, do not have a blood test to reflect the extent and severity of the disease. The EBDASI is a recently validated comprehensive scoring system developed to monitor the severity of EB. The scoring system is the first to take into account the activity of the disease, such as blistering and erosions, separately from the damage of the disease, including atrophy, scarring and pigmentation changes. Keeping these two categories separate is important because damage tends to be stable or get worse (very rarely gets better), but activity tends to vary and hence, change can be reliably monitored when evaluating new medications to treat the disease.

Methodology

The EBDASI looks at the disease in terms of four different regions of the body including the skin, scalp, mucous membrane, nails and other epithelialized areas. The skin is further broken down into 12 zones: the ears, face, neck, chest, abdomen, back, buttocks, arms, hands, legs, feet and anogenital areas. Each area is given a score out of 10 for activity and a value of either 1 or 0 for each of 6 different components of damage depending on if it is present or not. The EBDASI is a paper-based system that takes on average under ten minutes to complete.

Development

The EBDASI was developed and validated by Professor Dedee Murrell and her team of students and fellows at the St George Hospital, University of New South Wales, in Sydney, Australia. It was presented at the International Investigative Dermatology congress in Edinburgh in 2013 and published in the Journal of American Academy of Dermatology in 2014