Autoimmune bullous disorders

Abstract

Bullous skin diseases represent a group of organ-specific autoimmune disorders characterised by binding of circulating autoantibodies to adhesion molecules of the epidermis and the dermo-epidermal basement membrane zone. Binding of these autoantibodies to their antigenic targets results in loss of adhesion between epidermal keratinocytes and at the level of the basement membrane zone. Chronic blisters and secondary painful erosions are the clinical hallmark of autoimmune bullous disorders. Histopathology reveals the location of blister formation and helps to classify the subtype of the bullous skin disorder. Immunofluorescence is crucial for diagnosing autoimmune bullous skin disorders. Tissue-bound autoantibodies are detected by direct immunofluorescence of perilesional skin. Circulating autoantibodies can be visualised by indirect immunofluorescence using tissue substrates such as monkey oesophagus and sodium chloride-split human skin. Most of the autoantigens are available as recombinant proteins, which allows for autoantibody screening by ELISA or immunoblot analysis to confirm the primary diagnosis and, importantly, for immunoserological follow-up of patients.

Serological diagnosis of autoimmune bullous skin diseases: Prospective comparison of the BIOCHIP mosaic-based indirect immunofluorescence technique with the conventional multi-step single test strategy

“…Autoimmune bullous disorders are characterized by autoantibodies against desmosomal proteins (in pemphigus), adhesion molecules of the dermal-epidermal junction (in pemphigoid diseases), and epidermal/ tissue transglutaminase (in dermatitis herpetiformis), respectively [1-3]. The most frequent autoimmune bullous diseases are bullous pemphigoid and pemphigus, with incidences varying considerably between geographical regions [4-8].…”

Mucous membrane pemphigoid in a patient with hypertension treated with atenolol: a case report

“…We next considered the pemphigoid family, such as mucous membrane pemphigoid or cicatricial pemphigoid, because of the presence of oral mucosal lesions (generalized erythema, desquamation, multiple large ulcers covered with yellowish slough tissue along the gingiva, and multiple tense bullae of the oral mucosa and tongue). While bullous pemphigoid typically presents with skin lesions such as taut blisters, pruritus, papulovesicular or urticarial plaques on the flexor side of the extremities, oral mucosal involvement is uncommon [12]. After we observed the slight response of our patient to systemic steroids we then stopped prednisolone.…”

Autoimmune bullous skin diseases. Part 2: diagnosis and therapy

“…Depending on the location of the autoantigens intraepidemal or subepidermal loss of adhesion results which often but not always explains the clinical findings (Figure 1). Only the overall view of these tests can lead to the final diagnosis in the end (Table 1 and Figure 2) [1]. For a targeted securing of the diagnosis a generally valid step‐by‐step diagnostic approach is recommendable (Figure 3).…”