Bullous pemphigoid. Is it an immunologic disease?

Wm, Sams

doi:10.1001/archderm.102.5.485

Cited by 27 publications

(5 citation statements)

References 47 publications

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“…An increase in antigenicity which may be associa1ed \\ ith thi� could lead to the formation of ••auto anlibodic�" of the basemen1 mcmbranc LOne anti body typc. This agrees with the hypothc,i, of Sams ( 12) and Sams & Gleich ( 13). lhat thc basement mcmbrane has become antigenie, so thal specific basement membrane zone antibodie� are formed and bound there.…”

Section: Discussionsupporting

confidence: 84%

The activation of antigens of the basement membrane zone by proteolytic enzymes in vitro

Remy¹,

H²,

Stüttgen³

et al. 1976

Acta Derm Venereol

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Following brief exposure of cryostat sections of human skin to the action of proteolytic enzymes (papain or trypsin), there was a pronounced increase in the antibody binding sites of the basement membrane zone, shown in indirect immunofluorescence by an increased intensity in fluorescence of the basement membrane zone as compared with preparations which had not previously undergone incubation, and by an increase of 5-6 (papain) or 4-5 (trypsin) titre dilution steps. This effect was practically absent when guinea-pig tongue was used as the antigenic substrate. In conjunction with findings published in the literature, our results can be interpreted as indicating that the activation of the basement membrane zone antigens by proteolytic enzymes is associated with an increase in antigenicity which results in the formation of "auto-antibodies" of the basement membrane zone antibody type.

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Section: Discussionsupporting

confidence: 84%

The activation of antigens of the basement membrane zone by proteolytic enzymes in vitro

Remy¹,

H²,

Stüttgen³

et al. 1976

Acta Derm Venereol

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“…Collagen XVII (formerly known as BPAG2 or BP180) (COL17) is a transmembrane protein that plays a critical role in linking the cytoskeleton and the extracellular environment (Shimizu et al, 1989;Franzke et al, 2005). It is also an autoantigen in bullous pemphigoid, a blistering skin disease (Jablonska et al, 1958;Sams, 1970;Shimizu et al, 1989). Mutations in the human COL17 gene, COL17A1, lead to COL17 protein deficiency, reduced keratinocyte-basement membrane adhesion, and reductions in the size of hemidesmosome (HD) plaques, involved in epidermal adhesion (McMillan et al, 1998) (Zillikens and Giudice, 1999).…”

Section: Introductionmentioning

confidence: 99%

Collagen XVII Participates in Keratinocyte Adhesion to Collagen IV, and in p38MAPK-Dependent Migration and Cell Signaling

Qiao

Shibaki

Long

et al. 2009

Journal of Investigative Dermatology

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Collagen XVII (COL17) participates in keratinocyte adhesion and possibly migration, as COL17 defects disrupt keratinocyte-basal lamina adhesion and underlie the disease non-Herlitz junctional epidermolysis bullosa. Using small interference RNA (siRNA) to knock down COL17 expression in HaCaT cells, we assessed cell characteristics, including adhesion, migration, and signaling. Control and siRNA-transfected keratinocytes showed no difference in adhesion on plastic dishes after incubation for 8 hours in serum-free keratinocyte-growth medium; however, when grown on collagen IV alone or BD matrigel (containing collagen IV and laminin isoforms), COL17-deficient cells showed significantly reduced adhesion compared with controls (P<0.01), and mitogen-activated protein kinase (MAPK)/ERK kinase (MEK)1/2 and MAPK showed reduced phosphorylation. Furthermore, COL17-deficient HaCaT cells plated on plastic exhibited reduced motility that was p38MAPK-dependent (after addition of the p38MAPK inhibitor SB203580). Together, these results suggest that COL17 has significantly wider signaling roles than were previously thought, including the involvement of COL17 in keratinocyte adhesion to collagen IV, in p38MAPK-dependent cell migration, and multiple cell signaling events pertaining to MEK1/2 phosphorylation.

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“…17 An alternate pathogenetic hypothesis is that production of and linear deposition of an antibody to the basement membrane zone is a second¬ ary phenomenon that results follow¬ ing immunologie injury due to com¬ plement fixation by antigen-antibody complexes.…”

Section: Pathologic Findingsmentioning

confidence: 99%