Mucous Pemphigoid with Eye Involvement. A Serie of Cases

Czech version

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‘ workplace: 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: Čes-slov Derm, 99, 2024, No. 4, p. 164-173
Category: Pharmacologyand Therapy, Clinical Trials

Overview

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

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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

Labels

Dermatology & STDs Paediatric dermatology & STDs

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Article was published in

Czech-Slovak Dermatology

2024 Issue 4