TAILIEUCHUNG - Chapter 055. Immunologically Mediated Skin Diseases (Part 4)

Bullous Pemphigoid Bullous pemphigoid (BP) is a polymorphic autoimmune subepidermal blistering disease usually seen in the elderly. Initial lesions may consist of urticarial plaques; most patients eventually display tense blisters on either normalappearing or erythematous skin (Fig. 55-2). The lesions are usually distributed over the lower abdomen, groin, and flexor surface of the extremities; oral mucosal lesions are found in some patients. Pruritus may be nonexistent or severe. As lesions evolve, tense blisters tend to rupture and be replaced by erosions with or without surmounting crust. Nontraumatized blisters heal without scarring. The major histocompatibility complex class II allele HLA-DQβ1*0301. | Chapter 055. Immunologically Mediated Skin Diseases Part 4 Bullous Pemphigoid Bullous pemphigoid BP is a polymorphic autoimmune subepidermal blistering disease usually seen in the elderly. Initial lesions may consist of urticarial plaques most patients eventually display tense blisters on either normalappearing or erythematous skin Fig. 55-2 . The lesions are usually distributed over the lower abdomen groin and flexor surface of the extremities oral mucosal lesions are found in some patients. Pruritus may be nonexistent or severe. As lesions evolve tense blisters tend to rupture and be replaced by erosions with or without surmounting crust. Nontraumatized blisters heal without scarring. The major histocompatibility complex class II allele HLA-DQß 1 0301 is prevalent in patients with BP. Despite isolated reports several studies have shown that patients with BP do not have an increased incidence of malignancy in comparison with appropriately age- and gender-matched controls. Figure 55-2 Bullous pemphigoid with tense vesicles and bullae on erythematous urticarial bases. Courtesy of the Yale Resident s Slide Collection. Biopsies of early lesional skin demonstrate subepidermal blisters and histologic features that roughly correlate with the clinical character of the particular lesion under study. Lesions on normal-appearing skin generally show a sparse perivascular leukocytic infiltrate with some eosinophils conversely biopsies of inflammatory lesions typically show an eosinophil-rich infiltrate at sites of vesicle formation and in perivascular areas. In addition to eosinophils cell-rich lesions also contain mononuclear cells and neutrophils. It is not possible to distinguish BP from other subepidermal blistering diseases by routine histologic techniques alone. Direct immunofluorescence microscopy of normal-appearing perilesional skin from patients with BP shows linear deposits of IgG and or C3 in the epidermal basement membrane. The sera of 70 of these patients contain

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