TAILIEUCHUNG - Chapter 055. Immunologically Mediated Skin Diseases (Part 6)

Linear IgA Disease Linear IgA disease, once considered a variant form of dermatitis herpetiformis, is actually a separate and distinct entity. Clinically, these patients may resemble individuals with DH, BP, or other subepidermal blistering diseases. Lesions typically consist of papulovesicles, bullae, and/or urticarial plaques predominantly on central or flexural sites. Oral mucosal involvement occurs in some patients. Severe pruritus resembles that seen in patients with DH. Patients with linear IgA disease do not have an increased frequency of the HLA-B8/DRw3 haplotype or an associated enteropathy and hence are not candidates for treatment with a gluten-free diet. . | Chapter 055. Immunologically Mediated Skin Diseases Part 6 Linear IgA Disease Linear IgA disease once considered a variant form of dermatitis herpetiformis is actually a separate and distinct entity. Clinically these patients may resemble individuals with DH BP or other subepidermal blistering diseases. Lesions typically consist of papulovesicles bullae and or urticarial plaques predominantly on central or flexural sites. Oral mucosal involvement occurs in some patients. Severe pruritus resembles that seen in patients with DH. Patients with linear IgA disease do not have an increased frequency of the HLA-B8 DRw3 haplotype or an associated enteropathy and hence are not candidates for treatment with a gluten-free diet. The histologic alterations in early lesions may be virtually indistinguishable from those in DH. However direct immunofluorescence microscopy of normalappearing perilesional skin reveals linear deposits of IgA and often C3 in the epidermal basement membrane zone. Most patients with linear IgA disease demonstrate circulating IgA anti-basement membrane autoantibodies directed against neoepitopes in the proteolytically processed extracellular domain of BPAG2. These patients generally respond to treatment with dapsone 50-200 mg d. Epidermolysis Bullosa Acquisita EBA is a rare noninherited polymorphic chronic subepidermal blistering disease. The inherited form is discussed in Chap. 357. Patients with classic or noninflammatory EBA have blisters on noninflamed skin atrophic scars milia nail dystrophy and oral lesions. Because lesions generally occur at sites exposed to minor trauma classic EBA is considered to be a mechanobullous disease. Other patients with EBA have widespread inflammatory scarring and bullous lesions that resemble severe BP. Inflammatory EBA may evolve into the classic noninflammatory form of this disease. Rare patients present with lesions that predominate on mucous membranes. The HLA-DR2 haplotype is found with increased frequency in EBA

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