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Epidermolysis Bullosa
Epidermolysis bullosa (EB) is a rare genetic disorder featuring abnormally fragile epithelial tissue, blisters and erosions of the skin or mucous membranes following minor trauma or friction. As defects in skin integrity can lead to chronic and recurrent wounds, exposing patients to recurrent infections and potentially leading to unwanted fibrosis and deformities as well as skin cancer. The lesions are associated with pruritus and pain. Such complications and symptoms constitute a major burden of disease for patients.
Epidermolysis Bullosa Prurigo (EBP) is a rare subtype of dystrophic epidermolysis bullosa (DEB), therefore it is also known as itchy dystrophic epidermolysis bullosa. This subtype was first reported in 1994 by McGrath et al. Among them, eight patients presented with moderate macules or vesicles at or after birth, mainly on the extensor side of the extremities, predominantly on the anterior tibia, with heavy itching and frequent scratching. EBP is an autosomal dominant or recessive dermatosis caused by the COL7Al mutation, featuring intense pruritus and nodular itchiness, and also includes blistering and nail atrophy. The main symptoms of the patients in adulthood are numerous scratch marks, itchy rash-like papules, nodules and plaques on the front of the shins of the legs, but with few blisters, making them highly susceptible to misdiagnosis of various acquired pruritic skin diseases such as itchy nodular rash, neurodermatitis, hypertrophic lichen planus and artificial dermatitis. Clinically, it is particularly important in the treatment of this disease to improve or reduce the patient's intense pruritic symptoms and to treat the nodular rash to improve the signs.
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