Disorders of Skin Fragility

Epidermolysis bullosa and other related disorders of skin fragility represent a heterogeneous group of inherited disorders characterized by structural defects within the epidermis and dermo-epidermal junction. These disorders are characterized by recurrent blistering and erosions of trauma-prone sites with chronic wounds with a wide spectrum of clinical severity ranging from localized mild disease to severe life-threatening/ multisystem involvement. These disorders share overlapping features with autoimmune bullous disorders, warranting careful clinicopathological correlation and molecular testing for accurate diagnosis. Advances in molecular genetics have led to refined classification, improved diagnostic accuracy, and the development of targeted therapy.