Slizniční pemfigoid – naše zkušenosti
| Authors | |
|---|---|
| Year of publication | 2022 |
| Type | Conference abstract |
| MU Faculty or unit | |
| Citation | |
| Description | 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 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 laminin 332 and alpha6 beta4 integrin (ELISA only for BP antigens), in many cases it is not possible to make an unequivocal diagnosis. We organized data of patients with bullous erosive dermatosis suspected of mucous membrane pemphigoid treated at our clinic in the last 10 years. The diagnosis was determined on the basis of clinical features, immunofluorescence and histological examination. We enrolled 48 women and 10 men. The average age at the time of diagnosis was 64,3. Histology was positive in 24 patients, direct immunofluorescence was positive in 26 patients, indirect immunofluorescence in 27 patients and ELISA for BP antigens in 10 patients. Detection of laminin 332 antibodies was perfomed by Western blot analysis. Presence of several protein bans was detected, namely 100-120 kDa band in 12 sera, 140 kDa band in 1 serum and 60 kDa band in 15 sera. This database was created with the aim of creating a comprehensive overview of patients with mucous pemphigoid and to ensure their consistent management at the dermatovenerology clinic. Due to low sensitivity of available tests, diagnosis relies particularly on the clinical picture. Despite established therapy, we observe changes in disease activity and alternating periods of stabilization and progression. |