Autoimmune Responses in Patients with Linear IgA Bullous Dermatosis: Both Autoantibodies and T Lymphocytes Recognize the NC16A Domain of the BP180 Molecule

Lin, Mong-Shang; Fu, Chang-Ling; Olague-Marchan, Mónica; Hacker, Mary K.; Zillikens, Detlef; Giudice, George J.; Fairley, Janet A.

doi:10.1006/clim.2001.5177

Cited by 42 publications

(27 citation statements)

References 39 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Previous reports only identified serum autoantibody (10, 21, 33, 34) and autoaggressive T cells (23) recognizing epitopes within the NH 2 -terminal region of the BP180 ECD in the majority of BP patients. Specifically, these studies focused on T cell recognition of the NC16A subdomain, which resides within the NH 2 -terminal region of BP180 (24,25,27,36). In fact, a considerable number of BP and Pg patients studied displayed NC16A-specific peripheral T cell responses, which were mainly of the Th2 type or of a mixed Th1/Th2 type (24,25,27,36).…”

Section: Discussionmentioning

confidence: 99%

“…Specifically, these studies focused on T cell recognition of the NC16A subdomain, which resides within the NH 2 -terminal region of BP180 (24,25,27,36). In fact, a considerable number of BP and Pg patients studied displayed NC16A-specific peripheral T cell responses, which were mainly of the Th2 type or of a mixed Th1/Th2 type (24,25,27,36). Specifically, Lin et al (24) identified autoreactive T cells directed against the NC16A region of the BP180 ECD in 12 patients with active BP and two patients with Pg.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Autoreactive T and B Cells from Bullous Pemphigoid (BP) Patients Recognize Epitopes Clustered in Distinct Regions of BP180 and BP230

Thoma-Uszynski

Uter

Schwietzke

et al. 2006

The Journal of Immunology

108

View full text Add to dashboard Cite

Bullous pemphigoid (BP) is a well-characterized model of autoantibody-mediated autoimmunity, which presumably depends on autoreactive Th cells that promote the activation of autoreactive B cells. The two major autoantigens of BP are BP180 and BP230, two components of dermoepidermal adhesion complexes. Both, autoreactive Th cell responses and autoantibody profiles were characterized in 35 patients with acute onset BP using BP180 and BP230 proteins. Our findings indicate the following: 1) autoreactive Th cells recognized epitopes within the NH2-terminal (77.1%), COOH-terminal (65.7%), and central portion (57.1%) of the BP180 ectodomain; 2) IgG autoantibodies were found to exhibit similar or identical reactivity against the NH2-terminal (82.8%), COOH-terminal (77.1%), and central portion (37.1%) of the BP180 ectodomain; 3) T and B cell reactivity with the NH2-terminal portion of the BP180 ectodomain was associated with extensive BP, whereas the central portion was more frequently recognized in limited BP; 4) only 7 of 16 (43.7%) and 6 of 16 (37.5%) BP patients showed a Th cellular response against the COOH- and NH2-terminal regions of BP230, respectively, whereas 5) IgG reactivity against the COOH- and NH2-termini of BP230 was detected in 5 of 16 (31.3%) and 6 of 16 (37.5%) patients, respectively. These results demonstrate that Th and B cell reactivities against BP180, are, in contrast to BP230 reactivity, almost constantly detectable in BP patients, and differential epitope recognition of BP180 seems to be associated with distinct clinical severity. These observations support the concept that BP180, but not BP230, is the primary autoantigen of BP critical for disease development.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Autoreactive T and B Cells from Bullous Pemphigoid (BP) Patients Recognize Epitopes Clustered in Distinct Regions of BP180 and BP230

Thoma-Uszynski

Uter

Schwietzke

et al. 2006

The Journal of Immunology

108

View full text Add to dashboard Cite

show abstract

“…The incidence of LABD is estimated at 0.2-0.5 cases/1 million population/year. The disease most frequently develops in patients over the age of 60, although any age group may be affected [32,33].…”

Section: Linear Iga Bullous Dermatosis (Labd)mentioning

confidence: 99%

“…Częstość występowania szacuje się na 0,2-0,5 na milion rocznie. Najczęściej dotyczy osób po 60. roku życia, ale może pojawić się w każdej grupie wiekowej [32,33].…”

Section: Linear Iga Bullous Dermatosis (Labd)unclassified

Diseases presenting with vesicular and erosive lesions of oral mucous membranes

Ociepa¹,

Żebrowska²

2017

View full text Add to dashboard Cite

Disorders of the oral mucosa are an area of interdisciplinary cooperation involving physicians from a diverse range of medical specialties. Diseases characterized by a similar mucosal abnormalities include lichen planus, pemphigus vulgaris, paraneoplastic pemphigus, mucous membrane pemphigoid, epidermolysis bullosa acquisita, linear IgA bullous dermatosis and chronic ulcerative stomatitis. streszCzenie Choroby błony śluzowej jamy ustnej stanowią obszar interdyscyplinarnej współpracy lekarzy wielu specjalności. Do jednostek chorobowych o podobnym obrazie klinicznym zmian występujących na błonach ślu-zowych należą: liszaj płaski jamy ustnej, pęcherzyca zwykła, pęcherzy-ca paraneoplastyczna, pemfigoid błon śluzowych, nabyte pęcherzowe oddzielanie się naskórka, linijna IgA dermatoza pęcherzowa oraz przewlekłe wrzodziejące zapalenie jamy ustnej.Key words: lichen planus, pemphigus vulgaris, paraneoplastic pemphigus, mucous membrane pemphigoid, epidermolysis bullosa acquisita, chronic ulcerative stomatitis. Słowa kluczowe: liszaj płaski, pęcherzyca zwykła, pęcherzyca paraneo plastyczna, pemfigoid błon śluzowych, nabyte pęcherzowe oddzielanie się naskórka, przewlekłe wrzodziejące zapalenie jamy ustnej.

show abstract

“…A cellular response is also involved with complement activation, recruitment of inflammatory cells and release of proteolytic enzymes [17,18].…”

Section: Pathophysiology and Autoimmunitymentioning

confidence: 99%

Chronic Bullous Disease or Linear IgA Dermatosis of Childhood -Revisited

Patsatsi¹

2013

J Genet Syndr Gene Ther

View full text Add to dashboard Cite

Linear IgA dermatosis or chronic bullous disease of childhood (CBDC) is a nonhereditary, autoimmune subepidermal bullous disease, characterized by the presence of continuous linear deposits of IgA autoantibodies along the basement membrane zone. Antigens mainly involved in the pathogenesis of CBDC are a 97-kDa and a 120 -kDa protein, which represent fragments of the extracellular domain of collagen XVII (BP180), a transmembrane protein playing a critical role in maintaining the linkage between the intracellular and the extracellular structural elements involved in epidermal adhesion. Humoral and cellular response contributes to the pathogenetic mechanism leading to blister formation. CBDC is characterized by a clinical polymorphism in each patient, with the "cluster of jewels" pattern being the most typical clinical lesion. For the establishment of CBDC diagnosis, combination of histology and immunofluorescence studies are of utmost importance. The majority of patients respond to dapsone, sulfapiridine or systemic steroids in cases of widespread disease. CBDC tends to resolve spontaneously within several months to 5 years after its onset. In this review article, the key features of this rare autoimmune bullous disease are revisited.

show abstract

Autoimmune Responses in Patients with Linear IgA Bullous Dermatosis: Both Autoantibodies and T Lymphocytes Recognize the NC16A Domain of the BP180 Molecule

Cited by 42 publications

References 39 publications

Autoreactive T and B Cells from Bullous Pemphigoid (BP) Patients Recognize Epitopes Clustered in Distinct Regions of BP180 and BP230

Autoreactive T and B Cells from Bullous Pemphigoid (BP) Patients Recognize Epitopes Clustered in Distinct Regions of BP180 and BP230

Diseases presenting with vesicular and erosive lesions of oral mucous membranes

Chronic Bullous Disease or Linear IgA Dermatosis of Childhood -Revisited

Contact Info

Product

Resources

About