#PAGE_PARAMS# #ADS_HEAD_SCRIPTS# #MICRODATA#
preLekára.sk / Journals / Czech-Slovak Dermatology / 2024 - 4

Mucous Pemphigoid with Eye Involvement. A Serie of Cases

Česká verzia

Authors: L. Vojtěchová 1;  H. Jedličková 1;  I. Slaninová 2;  Z. Hayek 2;  T. Olejková 2;  D. Ivanišová 3;  L. Michalcová 3;  M. Horáčková 3
Authors place of work: I. dermatovenerologická klinika Lékařské fakulty Masarykovy univerzity a Fakultní nemocnice u sv. Anny v Brně, ERN, centrum pro vzácné puchýřnaté dermatózy, přednosta prof. MUDr. Hana Jedličková, Ph. D. 1;  Biologický ústav Lékařské fakulty Masarykovy univerzity, přednosta prof. RNDr. Ondřej Slabý, Ph. D. 2;  Oční klinika Fakultní nemocnice Brno, přednosta doc. MUDr. Oldřich Chrapek, Ph. D. 3
Published in the journal: Čes-slov Derm, 99, 2024, No. 4, p. 164-173
Category: Pharmacologyand Therapy, Clinical Trials

Summary

Mucous membrane pemphigoid is a rare autoimmune bullous disease with an annual incidence of 1 per million, typically starting at around 60 years of age. Clinical features include blisters and erosions with subsequent scarring, especially of the mucous membranes of the oral cavity, nose, eyes and genitals. Ocular pemphigoid is a severe type of mucous membrane pemphigoid affecting the conjunctiva. Due to the rarity of the disease, low sensitivity of standard investigations (histology and immunofluorescence investigation of tissue and serum) and unavailability of serological testing of laminin332 and α6β4 integrin (ELISA only for BP antigens), in many cases it is not possible to make an unequivocal diagnosis. Despite established therapy, we observe changes in disease activity and alternating periods of stabilization and progression.

We describe a cohort of patients with mucous membrane pemphigoid with ocular involvement treated at our clinic in 2021–2022. We organized data on the current patient population with a focus on age, clinical features, investigations performed, disease course and management. The diagnosis was determined on the basis of clinical features, immunofluorescence and histological examination. Simultaneously, some patients' sera were tested foranti-laminin332 and anti-β4integrin antibodies by western blot. We created this database with the aim of creating a comprehensive overview of patients with mucous pemphigoid and to ensure their consistent management at the dermatology and ophthalmology clinics.

Keywords:

autoimmune bullous disease – mucous membrane pemphigoid – ocular pemphigoid – clinical characteristics – immunology – diagnosis – therapy

Zdroje
  1. CHAN, L. S., AHMED, A. R., ANHALT G. J. et al. The first international consensus on mucous membrane pemphigoid: definition, diagnostic criteria, pathogenic factors, medical treatment, and prognostic indicators. Arch Dermatol, 2002, 138(3), p. 370–379. doi: 10.1001/archderm.138.3.370.
  2. CHEN, L. L., GABARRA, V., CHO, S. et al. Functional characterization of integrin α6β4 adhesion interactions using soluble integrin constructs reveals the involvement of different functional domains in the β4 subunit. Cell communication & adhesion, 2008, 15(4), p. 317–331. doi:10.1080/15419060802428356.
  3. CHUKWUMA, O., SAIKALY, S. K., MONTANEZ-WISCOVICH, M. et al. Ocular pseudopemphigoid with concomitant eyelid dermatitis secondary to rosacea. JAADCase Rep, 2020, 7, p. 62–64. doi: 10.1016/j. jdcr.2020.11.007
  4. DU, G., PATZELT, S., VAN BEEK, N., SCHMIDT, E. Mucous membrane pemfigoid. Autoimmun Rev, 2022, 21(4), p. 103036. doi: 10.1016/j.autrev.2022.103036.
  1. GEORGOUDIS, P., SABATINO, F., SZENTMARY, N. et al. Ocular Mucous Membrane Pemphigoid: Current State of Pathophysiology, Diagnostics and Treatment. Ophthalmol Ther, 2019, 8(1), p. 5–17. doi: 10.1007/s40123-019-0164-z.
  2. GOLETZ, S., ZILLIKENS, D., SCHMIDT, E. Structural proteins of the dermal-epidermal junction targeted by autoantibodies in pemphigoid diseases. Exp Dermatol, 2017, 26, p. 1154–1162. doi: 10.1111/exd.13446.
  3. HOFMANN, S. C., GÜNTHER, C., BÖCKLE, B. C. et al. S2k Guideline for the diagnosis and treatment of mucous membrane pemphigoid. J Dtsch Dermatol Ges, 2022, 20(11), p. 1530–1550. doi: 10.1111/ddg.14905.
  4. IVANIŠOVÁ, D., JEDLIČKOVÁ, H., VOJTĚCHOVÁ, L., HORÁČKOVÁ, M. Entropium jako jediný symptom očního jizvícího pemfigoidu. Chrapek O. et al. Oftalmologie v kazuistikách. Praha: EEZY Publishing, 2023, p. 130. ISBN: 978-80-88506-17-1.
  5. JEDLIČKOVÁ, H. Autoimunitní puchýřnatá onemocnění část II.: Subepidermální autoimunitní bulózní dermatózy. Čes-slov Derm, 2023, 98(4), p. 181–232.
  6. JOONES, V. A., PATEL, P. M., GIBSON, F. T. et al. The Role of Collagen XVII in Cancer: Squamous Cell Carcinoma and Beyond. Front Oncol, 2020, 10, p. 352. doi: 10.3389/fonc.2020.00352.
  7. KNEISEL, A., HERTL, M. Autoimmune bullous skin diseases. Part 2: diagnosis and therapy. J Dtsch Dermatol Ges, 2011, 9(11), p. 927–947. doi: 10.1111/j.1610-0387.2011.07809.x.
  8. KUMARI, S., BHOL, K. C., SIMMONS, R. K. et al. Identification of Ocular Cicatricial PemphigoidAntibody Binding Site(s) in Human β4 Integrin. Invest Ophthalmol Vis Sci, 2001, 42(2), p. 379–385.
  9. PATZELT, S., SCHMIDT, E. Autoimmunity against laminin 332. Front. Immunol, 2023, 14, 1250115. doi:10.3389/fimmu.2023.1250115.
  10. RASHID, H., LAMBERTS, A., BORRADORI, L. et al. European guidelines (S3) on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology – Part I. J Eur Acad Dermatol Venereol, 2021, 35(9), p. 1750–1764. doi: 10.1111/ jdv.17397.
  11. RASHID, H., MEIJER, J. M., BOLLING, M. C. et al. Insights into clinical and diagnostic findings as well as treatment responses in patients with mucous membrane pemphigoid: A retrospective cohort study. J Am Acad Dermatol, 2022, 87(1), p. 48–55. doi: 10.1016/j.jaad.2021.11.061.
  12. SCHMIDT, E., RASHID, H., MARZANO, A. V. et al. European Guidelines (S3) on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology – Part II. J Eur Acad Dermatol Venereol, 2021, 35(10), p. 1926–1948. doi: 10.1111/jdv.17395.
  13. SCHONBERG, S., STOKKERMANS, T. J. Ocular Pemphigoid. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing, 2023. Dostupné na www: https://www.ncbi.nlm.nih.gov/books/NBK 526100/.
  14. YOU, C., MA, L., ANES, S. D., STEPHEN, F. C. Longterm remission of ocularcicatricial pemphigoid off immunomodulatory therapy. Eur J Ophthalmol, 2018, 28, p. 157–162.

Publikace vznikla v rámci interního grantu podpořeného Fakultní nemocnicí u sv. Anny v Brně.

Autorka v souvislosti s tématem práce v posledních 12 měsících nespolupracovala s žádnou farmaceutickou firmou.

Do redakce došlo dne 11. 3. 2024.

Adresa pro korespondenci:
MUDr. Lucia Vojtěchová
I. dermatovenerologická klinika LF MU a FNUSA
Pekařská 664/53 602 00 Brno
e-mail: lucia.janickova@fnusa.cz

Štítky
Dermatology & STDs Paediatric dermatology & STDs
Článek Vaccination in Patients with Autoimmune Rheumatic Diseases
Článek Multiple Bluish Papules in Daughter and Mother. Minireview
Článek FORTHCOMING EVENTS
Článok vyšiel v časopise

Czech-Slovak Dermatology

Číslo 4
2024 Číslo 4
Najčítanejšie tento týždeň
Najčítanejšie v tomto čísle
Prihlásenie
Zabudnuté heslo

Zadajte e-mailovú adresu, s ktorou ste vytvárali účet. Budú Vám na ňu zasielané informácie k nastaveniu nového hesla.

Prihlásenie

Nemáte účet?  Registrujte sa

#ADS_BOTTOM_SCRIPTS#