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Müköz membran pemfigoidi

Year 2018, Volume: 1 Issue: 1, 1 - 9, 28.06.2018
https://doi.org/10.33204/mucosa.433950

Abstract

Müköz membran pemfigoidi (MMP),
pemfigoidlerin müköz membranları etkileyen alt tipidir. Klinik semptomlar ve
hedef antijenlere göre, oküler müköz membran pemfigoidi (OMMP) ve
anti-laminin332 MMP(anti-LN-332 MMP) gibi çeşitli alt tiplere ayrılmıştır.
Başlıca hedef antijen 180 kDa (BP180) olmakla birlikte, epidermal bazal membran
bölgesindeki (EBMZ) çeşitli yapısal proteinlere karşı otoantikorlar mevcuttur.
BP230, α6β4 integrin ve laminin 332 gibi diğer antijenler de, bu otoantikorlar
tarafından hedef alınabilmektedir. MMP klinik olarak, oral mukozada (%85),
konjuktivada (%65), daha az sıklıkta ise, burunda (%20-40), özefagusta (%5-15),
farinkste (%20), larinkste (%5-10) ve genitallerde (%20) erozyon ve büllerle
karakterizedir. MMP'nin klinik şiddeti, farklı klinik tiplerinde önemli
farklılıklar gösterir.OMMP ve anti-LN-332 MMP klinik tiplerinde aktif hastalık
uygun bir biçimde tedavi edilmediğinde körlük veya üst havayolu obstrüksiyonu
ile sonuçlanan ilerleyici skar gelişimi hastalığın ciddi bir komplikasyonudur.  Önceleri, MMP ile eş anlamlı olarak kullanılan
skatrisyel pemfigoid, şimdi skarla seyreden deri lezyonlarına sahip nadir
klinik fenotipi isimlendirmektedir. Klinik özelliklerdeki değişkenlikler ve
klinisyenler arasında aşinalığın azlığı, MMP tanısında, hasta ve doktor
kaynaklı gecikmelere yol açmaktadır. Doğru tanı için, direkt immünofloresan
mikroskopik inceleme ve serumda otoantikorların gösterilmesi zorunludur.
MMP'nin yönetimi ve prognozu hastalığın yaygınlığı ve şiddeti ile ilişkili olup
basamaklı bir yaklaşım ile, ilk seçenek olarak oral kortikosteroidleri (KS) ve
sıklıkla uzun dönem KS kullanımının yol açabileceği yan etkileri azaltmak için
adjuvanlarla kombinasyonu içerir.

References

  • 1. Chan LS, Ahmed AR, Anhalt GJ, et al. The first international consensus on mucous membrane pemphigoid: definition, diagnostic criteria, pathogenic factors, medical treatment, and prognostic indicators. Arch Dermatol 2002;138:370-9.
  • 2. Schmidt E, Zillikens D. Pemphigoid diseases. Lancet 2013;381:320-32.
  • 3. Egan CA, Lazarova Z, Darling TN, Yee C, Yancey KB. Anti-epiligrin cicatricial pemphigoid: clinical findings, immunopathogenesis, and significant associations. Medicine (Baltimore) 2003;82:177-86.
  • 4. Domloge-Hultsch N, Gammon WR, Briggaman RA, Gil SG, Carter WG, Yancey KB. Epiligrin, the major human keratinocyte integrin ligand, is a target in both an acquired autoimmune and an inherited subepidermal blistering skin disease. J Clin Invest 1992;90:1628-33.
  • 5. Terra JB, Pas HH, Hertl M, Dikkers FG, Kamminga N, Jonkman MF. Immunofluorescence serration pattern analysis as a diagnostic criterion in antilaminin-332 mucous membrane pemphigoid: immunopathological findings and clinical experience in 10 Dutch patients. Br J Dermatol 2011;165:815-22.

Mucous membrane pemphigoid

Year 2018, Volume: 1 Issue: 1, 1 - 9, 28.06.2018
https://doi.org/10.33204/mucosa.433950

Abstract

Mucous membrane pemphigoid (MMP) is the
subgroup of pemphigoid which affects mucous membranes. Several sub-types are
classified based on clinical symptoms and target antigens, such as ocular
mucous membrane pemphigoid (OMMP), localized vulvar pemphigoid (LVP) and
an-ti-laminin 332 MMP (anti-LN-332 MMP). Autoantibodies are directed against
various structural proteins in the epidermal basement membrane zone (EBMZ),
with the 180-kD antigen (BP180) as the main target antigen. Other antigens,
such as BP230, α6β4 integrin and laminin 332 can also be targeted by
autoantibodies. Clinically MMP is characterized by erosions and blistering of
the oral mucosa (85%), conjunctiva (65%), and less frequently, the nose
(20-40%), esophagus (5-15%), pharynx (20%), larynx (5-10%) and genitals (20%).
Clinical severity is highly variable in the different subtypes of MMP.
Progressive scar formation is a severe complication in active disease in OMMP
and anti-LN-332 MMP, resulting in blindness or upper airway obstruction when
not treated accurately. Previously, the term cicatricial pemphigoid was used
synonymously for MMP, however, at present the term refers to the rare clinical
phe-notype with scarring skin lesions. Patient and doctors delay is frequently
seen in MMP because of its variation in clinical presentation and unfamiliarity
among clinicians. For an accurate diagnosis, direct immunofluorescence
microscopy (DIF) and detection of circulating autoantibodies in serum is
mandatory. Management and prognosis of MMP depends on the severity and extent
of the disease and involves a stepwise approach with first choice treatment
with oral corticosteroids (CS), often used in combination with adjuvant reduce
the adverse effects caused by long-term CS use.

References

  • 1. Chan LS, Ahmed AR, Anhalt GJ, et al. The first international consensus on mucous membrane pemphigoid: definition, diagnostic criteria, pathogenic factors, medical treatment, and prognostic indicators. Arch Dermatol 2002;138:370-9.
  • 2. Schmidt E, Zillikens D. Pemphigoid diseases. Lancet 2013;381:320-32.
  • 3. Egan CA, Lazarova Z, Darling TN, Yee C, Yancey KB. Anti-epiligrin cicatricial pemphigoid: clinical findings, immunopathogenesis, and significant associations. Medicine (Baltimore) 2003;82:177-86.
  • 4. Domloge-Hultsch N, Gammon WR, Briggaman RA, Gil SG, Carter WG, Yancey KB. Epiligrin, the major human keratinocyte integrin ligand, is a target in both an acquired autoimmune and an inherited subepidermal blistering skin disease. J Clin Invest 1992;90:1628-33.
  • 5. Terra JB, Pas HH, Hertl M, Dikkers FG, Kamminga N, Jonkman MF. Immunofluorescence serration pattern analysis as a diagnostic criterion in antilaminin-332 mucous membrane pemphigoid: immunopathological findings and clinical experience in 10 Dutch patients. Br J Dermatol 2011;165:815-22.
There are 5 citations in total.

Details

Primary Language English
Subjects Clinical Sciences
Journal Section Review Articles
Authors

Jorrit Terra This is me

Joost Meijer This is me

Marcel Jonkman

Publication Date June 28, 2018
Published in Issue Year 2018 Volume: 1 Issue: 1

Cite

Vancouver Terra J, Meijer J, Jonkman M. Mucous membrane pemphigoid. Mucosa. 2018;1(1):1-9.