(1)
Misdiagnosis Association & Society, Seattle, WA, USA
Keywords
Pemphigus vulgarisDemosgleinAnti-desmogleinAutoantibodiesCutaneousMucosalMucocutaneousPemphigus Disease Area Index (PDAI)Autoimmune Bullous Skin Disorder Intensity Score (ABSIS)Pemphigus Vulgaris Activity Score (PVAS)Enzyme-linked immunosorbent assay (ELISA)Pemphigus foliaceusIndirect immunofluorescenceIn a study conducted by Daneshpazhooh et al. in 2014, the levels of anti-desmoglein (anti-DSG) 1 and 3 autoantibodies were tested in 73 patients, and they were compared with the clinical symptoms of the patients [1]. Anti-DSG1 was found in 56 (76.7%) of the patients, and anti-DSG3 was detected in 69 (94.5%) of the patients. Anti-DSG1 was also found in 48 (94.1%) of the patients with the mucocutaneous form of the disease, and anti-DSG3 was found in 50 patients (98%) with the mucocutaneous form.
DSG1 and DSG3 were detected in 2 (12.5%) and 15 (93.7%) of the patients with the mucosal form, respectively. DSG1 was detected in 6 (100%) and DSG3 was detected in 4 (66.7%) of the patients with the cutaneous form of the disease. The average levels of DSG1 in the patients with cutaneous, mucosal, and mucocutaneous forms of the diseases were 136.8 ± 28.5, 11.4 ± 3.3, and 131.4 ± 7.8, respectively (P < 0.001). Also, the average levels of DSG3 in the patients with cutaneous, mucosal, and mucocutaneous forms of the diseases were 117.3 ± 44.4, 236 ± 48, and 457.2 ± 26.2, respectively (P < 0.001). The extent of cutaneous involvement has a significant positive relationship with DSG1 (r-0.74 and P < 0.001). Additionally, a weak positive association was observed between the extent of cutaneous involvement and DSG3 (P < 0.001 and r-0.38).
In a study by Hallaji et al., 50 pemphigus vulgaris patients were investigated; 37 patients had the mucocutaneous form of the disease, 11 of them had the mucosal form of the disease, and 2 patients had the cutaneous form of the disease. Anti-desmoglein 3 and 1 sensitivity was 94% and 72%, respectively. Salivary sensitivity of anti-desmoglein 3 and 1 was 94% and 70%, respectively [2].
In a study by Rahbar et al., 100 pemphigus vulgaris patients were examined. Pemphigus Disease Area Index (PDAI) , Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) , and Pemphigus Vulgaris Activity Score (PVAS) along with desmoglein 1 and 3 levels were tested. The results show that a PDAI value greater than two is another criterion in the evaluation of the disease severity [3].
In the work of Saha et al., 95 pemphigus patients (79 pemphigus vulgaris and 16 pemphigus foliaceus ) were tested [4]. The results showed that the duration of the disease is significantly longer in Indo-Asian patients in comparison with Caucasian-British patients. Also, young age at the time of disease onset was accompanied with bad prognosis. The average age of the patients at the time of disease onset among the patients whose disease lasted less than 5 years was 49, and among the patients whose disease lasted more than 5 years, the age of symptoms onset was 40 years (p = 0.03). High titers of antibodies at the time of disease onset were accompanied with a longer time period to remission. Furthermore, high levels of desmoglein 3 at the beginning of examination resulted in longer duration of the disease [4].
Harman et al. examined the relationship between anti-DSG1 and 3 autoantibody levels with the severity of pemphigus vulgaris in 2001 [5]. Enzyme-linked immunosorbent assay (ELISA) was performed on 424 serum samples from 80 pemphigus vulgaris and 24 pemphigus foliaceus patients. The anti-DSG1 level had strong correlation with severity of cutaneous involvement, while anti-DSG3 levels had a relationship with the extent of mucosal involvement. Anti-DSG1 levels, even after adjustment for the effect of anti-DSG3, had no relationship with oral involvement [5].