Underrecognition of the Heterogeneous Clinical Spectrum of Bullous Pemphigoid

Hertl, Michael; Schmidt, Thomas

doi:10.1001/jamadermatol.2013.4250

Cited by 5 publications

(3 citation statements)

References 14 publications

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“…The most common clinical subtype of pemphigoid diseases is classical generalized BP, characterized by tense blisters on erythematous skin on the trunk and extremities ( 2 , 17 ). Other rarer variants, such as localized BP, mucous membrane pemphigoid, anti-laminin-gamma-1 pemphigoid, and childhood BP, have been also described ( 18 , 19 ).…”

Section: Introductionmentioning

confidence: 99%

Autoreactive Peripheral Blood T Helper Cell Responses in Bullous Pemphigoid and Elderly Patients With Pruritic Disorders

et al. 2021

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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.

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Section: Introductionmentioning

confidence: 99%

Autoreactive Peripheral Blood T Helper Cell Responses in Bullous Pemphigoid and Elderly Patients With Pruritic Disorders

et al. 2021

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“…BP results from autoantibody mediated subepidermal blister formation typically associated with significant pruritus. Several retrospective studies suggest that at least 20% of all BP cases present with a nonbullous phenotype . Occasionally, the bullous disease remains localized to the pretibial area or other specific body regions .…”

Section: Introductionmentioning

confidence: 99%

“…Several retrospective studies suggest that at least 20% of all BP cases present with a nonbullous phenotype. 2,3 Occasionally, the bullous disease remains localized to the pretibial area or other specific body regions. 4 Oral mucosa involvement is seen in 10-20% of the patients, while other mucosal areas are rarely affected.…”

Section: Introductionmentioning

confidence: 99%

Failure of initial disease control in bullous pemphigoid: a retrospective study of hospitalized patients in a single tertiary center

et al. 2017

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The low frequency of recalcitrant BP (6.1%) and the rarity of involvement of the oral mucosa (1.8%) in this study suggest that Israeli BP patients present a milder phenotype compared to similar patients from other geographic areas. Hypertension, head involvement, and metformin therapy were found to be significantly associated with the need for adjuvant therapy to achieve initial control, suggesting that these parameters may serve as predictors of treatment response in BP.

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Nonbullous pemphigoid: A systematic review

Lamberts

Meijer

Jonkman

2018

Journal of the American Academy of Dermatology

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Underrecognition of the Heterogeneous Clinical Spectrum of Bullous Pemphigoid

Abstract: Bullous pemphigoid (BP) is commonly characterized clinically by the presence of tense blisters that develop on erythematous or urticarial pruritic skin. To establish the diagnosis of BP, the presence of tissue-

Cited by 5 publications

References 14 publications

Autoreactive Peripheral Blood T Helper Cell Responses in Bullous Pemphigoid and Elderly Patients With Pruritic Disorders

Autoreactive Peripheral Blood T Helper Cell Responses in Bullous Pemphigoid and Elderly Patients With Pruritic Disorders

Failure of initial disease control in bullous pemphigoid: a retrospective study of hospitalized patients in a single tertiary center

Nonbullous pemphigoid: A systematic review

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