The association of HLA-DQ7 with bullous pemphigoid is restricted to men

Banfield, CC; Wojnarowska, Fenella; Allen, J.; George, Sandhya; Va, Venning; Ki, Welsh

doi:10.1046/j.1365-2133.1998.02350.x

Cited by 48 publications

(40 citation statements)

References 16 publications

(21 reference statements)

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“…35 DQB1*0301 has also been reported in association with the closely related disorder BP, in which circulating autoantibodies target similar BMZ antigens. 16,36 Sarcoidosis has been associated with DQB1*0301-bearing haplotypes, 37 reactive arthritis with HLA-B27/ DRB1*0408/DQB1*0301, 38 alopecia areata with DRB1*1104 and DQB1*0301 39 and lichen sclerosus with DR4/DQ7. 40 In conclusion, our study confirms that the majority of patients with MMP share a common genetic predisposition.…”

Section: Discussionmentioning

confidence: 99%

Mucous membrane pemphigoid: HLA-DQB1*0301 is associated with all clinical sites of involvement and may be linked to antibasement membrane IgG production

Setterfield¹,

Theron²,

Vaughan³

et al. 2001

Br J Dermatol

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The DQB1*0301 allele confers a predisposition to all subgroups of MMP and may have a role in T-cell recognition of basement membrane antigens, resulting in the production of anti-BMZ IgG autoantibodies. The positive trend between increased allelic expression of DQB1*0301 in patients with ocular disease and in those with a higher clinical score, further suggests a role for this allele in disease severity.

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Section: Discussionmentioning

confidence: 99%

Mucous membrane pemphigoid: HLA-DQB1*0301 is associated with all clinical sites of involvement and may be linked to antibasement membrane IgG production

Setterfield¹,

Theron²,

Vaughan³

et al. 2001

Br J Dermatol

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“…Another study found that the association of BP with DQβ1*0301 was restricted to men (44). In our own experience, several BP180-specific Th1 and Th2 cell clones derived from BP patients were found to be restricted by HLA-DQβ1*0301 (45).…”

Section: Autoreactive T Lymphocytes In the Pemphigoidsmentioning

confidence: 94%

T cell control in autoimmune bullous skin disorders

Hertl¹

2006

Journal of Clinical Investigation

198

129

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Autoimmune bullous disorders are a group of severe skin diseases characterized clinically by blisters and erosions of skin and/or mucous membranes. A hallmark of these disorders is the presence of IgG and occasionally IgA autoantibodies that target distinct adhesion structures of the epidermis, dermoepidermal basement membrane, and anchoring fibrils of the dermis. This Review focuses on the potential role of autoreactive T cells in the pathogenesis of these disorders. Pemphigus vulgaris (PV) and bullous pemphigoid (BP) are the best-characterized bullous disorders with regard to pathogenesis and T cell involvement. Activation of autoreactive T cells in PV and BP is restricted by distinct HLA class II alleles that are prevalent in individuals with these disorders. Autoreactive T cells are not only present in patients but can also be detected in healthy individuals. Recently, a subset of autoreactive T cells with remarkable regulatory function was identified in healthy individuals and to a much lesser extent in patients with PV, suggesting that the occurrence of autoimmune bullous disorders may be linked to a dysfunction of Tregs.Autoimmune bullous skin disorders are characterized by the presence of autoantibodies that target distinct adhesion molecules of the epidermis and dermoepidermal basement membrane zone, leading to a loss of adhesive function of the target antigen(s) and, clinically, to the appearance of blisters and erosions (1, 2) (Figures 1 and 2). Desmogleins are transmembranous components of desmosomes, adhesion units specialized in conferring epidermal keratinocyte cohesion and linked to intercellular molecules of the desmosomal plaque, which in turn interact with components of the cytoskeleton (Figure 1). In pemphigus, IgG autoantibodies against desmoglein 3 (Dsg3) and Dsg1 lead to loss of desmosomal adhesion of epidermal keratinocytes and intraepidermal blister formation (Figure 2). In the pemphigoids, IgG autoantibodies against components of the dermoepidermal basement membrane such as bullous pemphigoid (BP) antigen 180 (BP180; also referred to as BP antigen 2 and type XVII collagen), BP antigen 230 (BP230; also referred to as BP antigen 1), and laminin 5 interfere with the adhesion of basal epidermal keratinocytes to the dermoepidermal basement membrane zone (Figure 1). BP180 and BP230 are transmembranous and intracellular components, respectively, of hemidesmosomes of basal epidermal keratinocytes, while laminin 5 is a ligand of BP180 located in the lamina lucida of the basement membrane zone (Figure 1). The autoantigen of epidermolysis bullosa, type VII collagen, is the major component of anchoring fibrils that connect the dermoepidermal basement membrane with interstitial collagen bundles of the dermis (Figure 1). The autoantigen of dermatitis herpetiformis, epidermal transglutaminase, is targeted by autoantibodies of the IgA class in the papillary dermis (Figure 2).Based on the specificity of the targeted antigens, several clinically and immune serologically distinct bullous disorders have be...

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“…Formation of numerous milia seems to be relatively rare in BP, although several cases of BP with milium formation during recovery have been reported . Recently, Tsuruta et al .…”

Section: Discussionmentioning

confidence: 99%

Refractory bullous pemphigoid leaving numerous milia during recovery

Uchida

Oiso

Koga

et al. 2014

The Journal of Dermatology

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Recovery with milia may occur in bullous pemphigoid (BP), mucous membrane pemphigoid (MMP) and epidermolysis bullosa acquisita (EBA). Scarring commonly occurs in MMP and EBA. Here, we report a 62-year-old man patient with BP, who was left with numerous milia during recovery. The patient had immunoglobulin (Ig)G autoantibodies to the recombinant protein of the BP180-NC16a domain and the soluble 120-kDa ectodomain of BP180 (linear IgA bullous dermatosis [LAD]-1). There are cases of BP with IgG autoantibodies to LAD-1 and/or the recombinant protein of BP180 C-terminal domain. Extensive milia formation during recovery may be associated with immunological predisposition and/or improper interaction between hemidesmosomes and the extracellular matrix components.

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The association of HLA-DQ7 with bullous pemphigoid is restricted to men

Cited by 48 publications

References 16 publications

Mucous membrane pemphigoid: HLA-DQB1*0301 is associated with all clinical sites of involvement and may be linked to antibasement membrane IgG production

Mucous membrane pemphigoid: HLA-DQB1*0301 is associated with all clinical sites of involvement and may be linked to antibasement membrane IgG production

T cell control in autoimmune bullous skin disorders

Refractory bullous pemphigoid leaving numerous milia during recovery

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