The Dystrophic Epidermolysis Bullosa Research Association has released comprehensive guidelines on the diagnosis and treatment of squamous cell carcinoma (SCC) in individuals with EB.

Among the recommendations:

• Conduct full-skin examinations every 3 to 6 months from as early as 10 years of age in those with recessive dystrophic EB, and from 20 years of age in the lower-risk groups.

• Wide local excision with a 2-cm excision margin is the treatment of choice. Additional options include limb amputation and more minimally invasive surgical techniques.

• Involve the patient in deciding which surgical option to choose.

• Use radiotherapy, chemotherapy, cetuximab, and tyrosine kinase inhibitors palliatively, though none have a role as definitive treatments.

• Preventative measures include retinoids for high-risk patients.