Clinical Relevance of Autoantibodies in Patients with Autoimmune Bullous Dermatosis

Abstract

The authors present their experience related to the diagnosis, treatment, and followup of 431 patients with bullous pemphigoid, 14 patients with juvenile bullous pemphigoid, and 273 patients with pemphigus. The detection of autoantibodies plays an outstanding role in the diagnosis and differential diagnosis. Paraneoplastic pemphigoid is suggested to be a distinct entity from the group of bullous pemphigoid in view of the linear C3 deposits along the basement membrane of the perilesional skin and the “ladder” configuration of autoantibodies demonstrated by western blot analysis. It is proposed that IgA pemphigoid should be differentiated from the linear IgA dermatoses. Immunosuppressive therapy is recommended in which the maintenance dose of corticosteroid is administered every second day, thereby reducing the side effects of the corticosteroids. Following the detection of IgA antibodies (IgA pemphigoid, linear IgA bullous dermatosis, and IgA pemphigus), diamino diphenyl sulfone (dapsone) therapy is preferred alone or in combination. The clinical relevance of autoantibodies in patients with autoimmune bullous dermatosis is stressed.

Comparative Study of Direct Immunofluorescence in Discoid Lupus Erythematosus and Bullous Pemphigoid

“…This was comparable with the results of previous studies on DIF of lesional or perilesional skin of patients with DLE, which demonstrated that IgM deposition at the epidermal BMZ was the most frequent immunoreactant. 3,4 IgG and C3 depositions at the epidermal BMZ were markedly more frequent in BP, as previously reported, 5 although these may also be relatively frequent in DLE. The number of positive immunoreactants at the epidermal BMZ may be more contributory in discriminating DLE from BP.…”

“…4 In contrast, direct immunofluorescence (DIF) in BP most frequently reveals linear depositions of IgG and C3 in the epidermal BMZ. 5 Closer examination of DIF in BP shows true linear or n-serrated linear pattern but never shows u-serrated pattern. 6 Some patients with lupus erythematosus have autoantibodies to BP.…”

Immune‐mediated diseases: what can be found in the oral cavity?

“…Bullous pemphigoid is a blistering disease affecting predominantly older individuals . It is clinically characterized by generalized, pruritic tense blisters, and crusts, usually in erythematous or apparently normal skin, together with infiltrated and urticarial plaques, papules, or eczematous lesions.…”

“…Generalized pemphigoid is the most common form of the disease, with dozens to hundreds of blisters, usually affecting the elderly. The localized form is characterized by some solitary eruptions on the head or on the extensor surface of the extremities, without causing complaints . The histological features of bullous pemphigoid include subepidermal blisters with inflammatory infiltrates that often are rich in eosinophils but also contain lymphocytes, histiocytes, or neutrophils.…”

“…The histological features of bullous pemphigoid include subepidermal blisters with inflammatory infiltrates that often are rich in eosinophils but also contain lymphocytes, histiocytes, or neutrophils. These can also be observed in several other related conditions, and therefore further diagnostic testing is essential . Biochemical characterization of the bullous pemphigoid antigen has shown the existence of two bullous pemphigoid antigens, BP230 for the 230 kDa protein and BP180 for the 180 kDa protein, both located next to the hemidesmosomes …”