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By Lawrence S Chan

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Extra resources for Blistering Skin Diseases

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Pemphigus vegetans needs to be differentiated from the heritable Hailey–Hailey disease and a subepidermal blistering disease, pemphigoid vegetans—which has a very similar clinical phenotype—by histopathological and immunopathological tests. In addition, patients with paraneoplastic pemphigus can present with the clinical phenotype of pemphigus vegetans. 16 A pemphigus vegetans lesion on a patient’s nose. Differential diagnoses The differential diagnosis can be any of the following: pemphigoid vegetans; paraneoplastic pemphigus; or Hailey–Hailey disease (familial benign pemphigus).

A de novo autoantigen-induced active animal model of BP, which is not currently available, may help answer this question in full. In a 2008 article, investigators showed that the presence or level of IgE class anti-BP180, but not IgE antiBP230, was associated with more extensive skin lesions, thus supporting the role of BP180, and possibly also IgE [122a]. The pathogenic role of BP180 is further supported by a mouse model in which transgenic mouse skin containing human BP180 engrafted onto syngeneic wild-type mice elicited strong anti-BP180 antibody response and blistering phenotype [131b].

42 Clinical features Paraneoplastic pemphigus (PNP) is an autoimmune mucocutaneous disease associated with a neoplasm, either benign or malignant. Paraneoplastic pemphigus has a distinct clinical phenotype, differing from that of the classic IgG-mediated pemphigus vulgaris [108; 115]. The PNP phenotype is quite variable and polymorphic, consisting of a mixture of blisters, erosions, papules, and targetoid lesions. In the first report of the disease where PNP is defined, patients suffered pruritic blisters that ruptured easily, involving upper trunk, head and neck, and proximal extremities [108].

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