WHAT IS EPIDERMOLYSIS BULLOSA?
Epidermolysis Bullosa (EB) is a rare group of inherited disorders that manifests as blistering or erosion of the skin and, in some cases, the epithelial lining of other organs, in response to little or no apparent trauma
Oma suffers from Dystrophic Epidermolysis Bullosa (DEB), a type of EB
PREVALENCE
While the prevalence in India is not documented, the exact prevalence of EB is unknown worldwide. Mild variants have been estimated to occur as frequently as 1 per 50,000 births. The more severe varieties like that of Oma are believed to occur in 1 per 500,000 births annually
CARE
The treatment of EB is primarily preventive and supportive. Surgical procedures can correct the deformities caused by repeated episodes of blistering and scarring of the hand. Patients with limited donor sites for a skin graft may need advanced therapy with bio-engineered skin products that are an alternative to skin graft
A water mattress and soft fleece covering help to limit the friction and trauma that lead to blistering Keeping the palms and soles cool and dry during hotter weather helps to minimise blistering, Preventive measures are the key to successful management of EB. The condition requires cooperation of patients, parents, and physicians caring for the patient
Taping bandages directly to the skin must be avoided Medical surveillance for involvements of kidney, urinary tract, eye, and GI tract should become part of the routine evaluation of children and adults with EB.