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Mechanobullous Disorders

»	What are the mechanobullous disorders?
»	Do all of the blisters that form in EB develop in the same layer of the skin?
»	How is EB classified, and what are the major modes of inheritance?
»	Describe the clinical findings in EB simplex.
»	Is the cause of EB simplex known?
»	What are the clinical findings in junctional EB?
»	Is the cause of junctional EB known?
»	What are the clinical findings in dystrophic EB?
»	Is the cause of dystrophic EB known?
»	What is Kindler’s syndrome?
»	Can a definitive diagnosis of the various EB types be made on clinical presentation?
»	Are there any treatments for the mechanobullous disorders?
»	Are prenatal diagnostic techniques useful for this group of disorders?

Do all of the blisters that form in EB develop in the same layer of the skin?

Fig. 6.1 Layers of the skin with major sites of splitting in epidermolysis bullosa (EB). (Courtesy of James E. Fitzpatrick, MD.)

No. Depending on the type of EB there are three basic levels of separation within the skin. The two basic layers of skin are the epidermis and dermis. The junction between these two layers contains important structural proteins and is called the dermal–epidermal junction (DEJ) (Fig. 6-1). Separation due to trauma may occur in any one of these three anatomic sites.