Are there acquired blistering diseases of the BMZ and dermis?

Yes. There are several diseases in which blistering occurs secondary to disruption of the structures of the BMZ and dermis. As with the epidermal blistering diseases, antibodies to the hemidesmosomes and other structures within the BMZ and dermis cause separation of the skin and blistering. In the uppermost portion of the BMZ, the LL, hemidesmosomes bind the basal keratinocytes to the basement membrane. Bullous pemphigoid (BP) is a classic example of an acquired blistering disease in which antihemidesmosomal antibodies are produced and appear to induce inflammation and subsequent damage of the hemidesmosomes, causing a blister to develop between the cells and the basement membrane.




A partial list of the skin diseases associated with antibodies and damage to the basement membrane structures and dermis are listed in Table 1-2.

Table 1-2. Skin Diseases Associated with Antibodies and Damage to Basement Membrane Structures and Dermis
	DISEASE		CLINICAL APPEARANCE AND LOCATION		BASEMENT MEMBRANE MOLECULE INVOLVED
	Bullous pemphigoid		Tense blisters diffusely		BP antigens 1 (BP230) and 2 (BP180)
	Pemphigoid (herpes) gestationis		Urticarial blisters with pruritus in late pregnancy		BP antigen 2 (BP180 or collagen XVII antigen)
	Epidermolysis bullosa acquisita		Friable skin and blistering knees, elbows, and sites of increased pressure		Type VII dermal collagen (anchoring fibril antigen)
	Bullous lupus erythematosus		Blistering face and trunk with flairs of systemic lupus erythematosus (SLE)		Type VII dermal collagen and lamins 5 and 6
	Linear IgA bullous disease (LIBD)		Tense vesicles in annular and target-like patterns on the trunk		BP antigen 2 (BP180 or collagen XVII antigen) and 97 kDa