Abstract
Bullous pemphigoid (BP) is a prototypic autoimmune disorder of the elderly, characterized by serum IgG autoantibodies, namely anti-BP180 and anti-BP230, directed against components of the basal membrane zone that lead to sub-epidermal loss of adhesion. Pruritus may be indicative of a pre-clinical stage of BP, since a subset of these patients shows serum IgG autoantibodies against BP230 and/or BP180 while chronic pruritus is increasingly common in the elderly population and is associated with a variety of dermatoses. Clinical and experimental evidence further suggests that pruritus of the elderly may be linked to autoimmunity with loss of self-tolerance against cutaneous autoantigens. Thus, the objective of this study was to determine autoreactive T cell responses against BP180 in elderly patients in comparison to patients with BP. A total of 22 elderly patients with pruritic disorders, 34 patients with bullous or non-bullous BP and 34 age-matched healthy controls were included in this study. The level of anti-BP180 and anti-BP230 IgG serum autoantibodies, Bullous Pemphigoid Disease Area Index (BPDAI), and pruritus severity were assessed for all patients and controls. For characterization of the autoreactive T cell response, peripheral blood mononuclear cells were stimulated ex vivo with recombinant BP180 proteins (NH2- and COOH-terminal domains) and the frequencies of BP180-specific T cells producing interferon-γ, interleukin (IL)-5 or IL-17 were subsequently determined by ELISpot assay. Patients with BP showed a mixed Th1/Th2 response against BP180 while autoreactive Th1 cells were identified in a minor subset of elderly patients with pruritic disorders. Furthermore, our T cell characterization revealed that therapeutic application of topical clobetasol propionate ointment in BP patients significantly reduced peripheral blood BP180-specific T cells, along with clinically improved symptoms, strongly suggesting a systemic immunosuppressive effect of this treatment.
Highlights
Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering skin disease, which usually affects people in the 6th to 8th decade of life and is clinically characterized by bullous and non-bullous skin lesions [1,2,3]
Urticarial plaques, and severity of pruritus were increased in elderly patients (EP) with pruritic disorders and non-bullous pemphigoid (nBP) patients compared to healthy controls (HC)
IgG autoantibodies against BP180 and BP230 have already been described in a subset of EP with pruritic disorders [23, 39] which in contrast to classical BP do not show IgG- and/or C3-deposits at the dermal-epidermal basement membrane zone (BMZ) [4]
Summary
Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering skin disease, which usually affects people in the 6th to 8th decade of life and is clinically characterized by bullous and non-bullous skin lesions [1,2,3]. Since an intense pruritus is present in BP, it has been thought that pruritic disorders of the elderly may represent a prodromal stage of BP [4,5,6]. Pruritus frequently occurs in the elderly population [7] with a worldwide prevalence between 7.3% and 41% [8,9,10]. Immunological alterations, like immune senescence and increased loss of tolerance to self-antigens, can play a key role in the etiology of pruritus of the elderly [10, 12]. A reduction of CD8+ T cells has been reported in elderly patients (EP) with pruritic disorders, which further supports the concept of an agedependent CD4+ Th2 polarization as a potential factor to the development of pruritus [4, 13, 16]
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