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[Anti-p200 pemphigoid].

M M Holtsche1, S Goletz2, D Zillikens2

  • 1Klinik für Dermatologie, Allergologie und Venerologie, Universität zu Lübeck, Ratzeburger Allee 160, 23538, Lübeck, Deutschland. maikemarleen.holtsche@uksh.de.

Der Hautarzt; Zeitschrift Fur Dermatologie, Venerologie, Und Verwandte Gebiete
|March 15, 2019
PubMed
Summary
This summary is machine-generated.

Anti-p200 pemphigoid is a rare autoimmune blistering disease. Diagnosis involves skin biopsy, autoantibody detection, and reactivity with a 200 kDa protein (p200), though its identity remains unknown.

Keywords:
AutoantibodiesBasement membrane zoneBlisterImmunofluorescenceImmunosuppression

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Area of Science:

  • Dermatology
  • Autoimmunology
  • Molecular Biology

Background:

  • Anti-p200 pemphigoid is a rare autoimmune blistering disease first described in 1996.
  • It shares similarities with bullous pemphigoid but affects younger patients with lesions on hands and feet.
  • Laminin γ1 was identified as a target antigen in most cases, but its pathogenic role is unclear.

Purpose of the Study:

  • To summarize the diagnostic criteria and clinical features of anti-p200 pemphigoid.
  • To highlight the ongoing research into the molecular identity of the p200 antigen.
  • To discuss current therapeutic approaches for anti-p200 pemphigoid.

Main Methods:

  • Diagnosis relies on direct and indirect immunofluorescence microscopy of skin biopsies.
  • Immunoblotting is used to detect autoantibodies against a 200 kDa protein (p200).
  • Patient sera are tested for reactivity with laminin γ1 and p200.

Main Results:

  • The gold standard for diagnosis combines direct immunofluorescence, indirect immunofluorescence on salt-split skin, and immunoblotting for p200 reactivity.
  • Laminin γ1 is recognized by antibodies in 90% of patients, but lacks direct pathogenic relevance.
  • The molecular identity of the p200 antigen remains to be elucidated.
  • Clinical presentation is heterogeneous, often resembling bullous pemphigoid but with younger onset and acral lesions.

Conclusions:

  • Anti-p200 pemphigoid diagnosis requires a multi-faceted approach including immunofluorescence and immunoblotting.
  • Further research is needed to identify the p200 antigen and clarify the role of laminin γ1.
  • Treatment involves corticosteroids, dapsone, and immunosuppressants.