About Epidermolysis Bullosa

Symptoms for all EBS Subtypes:^2-4

• Blistering happens in the upper layer of the skin causing erosions and crusts.
• Fragile skin that blisters easily, especially on the palms of the hands and the soles of the feet.
• Hot, red, itchy, and painful skin.
• Blisters heal with hyper- or hypo-pigmentation (lightening or darkening of the skin).
• Blistering is made worse by heat, humidity, and sweating.
• Hyperhidrosis (excessive sweating) primarily of the feet.
• Blistering tends to decrease during adolescence and becomes limited to the hands and feet.
• During infancy, milia (small white cysts) and EB naevi (birthmarks or moles) are common.
• Palmoplantar keratoderma (the thickening of the skin on the palms of the hands and soles of the feet) develops gradually, which can be painful and reduce mobility.

Additional symptoms for Severe EBS:^2,3,5

• Blistering tends towards the upper and lower extremities, face, and torso.
• Hemorrhagic blisters are common.
• Nails may be thick and dystrophic (appear damaged, misshapen, discolored, and curvy).
• Herpetiform blistering pattern (appear in clusters).
• Oral and esophageal blisters and erosions can occur and may interfere with feeding during infancy and early childhood. Mucosal blistering usually improves with age.
• Vocal hoarseness may develop due to laryngeal stenoses or strictures (which narrows the laryngeal airway). This is not life threatening but should be managed by a medical professional.
• Dysphagia (trouble swallowing), constipation, and other gastrointestinal issues are uncommon.
• Neonatal complications associated with severe EBS can be life threatening in the first year of life.