Epidermolysis bullosa simplex · EBS-PLEC · Plectin-deficient EBS · Skin fragility syndrome · Hautblasen-Krankheit
This is an inherited skin disorder caused by a deficiency in plectin, a structural protein that helps bind the layers of the skin together. Without enough functional plectin, the skin lacks its normal structural integrity and becomes extremely fragile. Even minor friction, pressure, or rubbing causes the outer layers of the skin to separate and form painful blisters.
Symptoms usually become visible during puppyhood. Owners will notice blisters, open sores, and crusts developing on areas prone to friction, such as the paw pads, ears, elbows, and inside the mouth, especially after physical activity.
There is no cure for this condition, requiring lifelong supportive care focused on wound management, pain relief, and preventing secondary skin infections. Managing this severe disease is highly demanding and typically costs between €3,000 and €12,000 over the dog's lifetime.
Estimated range of typical treatment cost. Actual cost depends on severity, clinic and region.
More conditions affecting the Skin & coat.