(1)
Misdiagnosis Association & Society, Seattle, WA, USA
Keywords
Pemphigus vulgarisDesmogleinPemphigus Disease Area Index (PDAI)Mucosal remissionCutaneous remissionFifty-two pemphigus vulgaris patients were investigated in the author’s previously unpublished study. The average age of the patients was 46 (±13.6) years, and the average weight of the patients was 72 (±14.9) kg. Of the patients, 29 were male (55.8%) and 23 of them (44.2%) were female (Fig. 14.1).
Fig. 14.1
Sex frequency of patients: 29 of the patients (58% of the patients) were males, and 23 patients (44.2% of the patients) were females (Source: Pooya Khan Mohammad Beigi)
Four cases (7.7%) had the cutaneous form, only 7 cases had the mucosal type (13.5), and 41 cases had the mucocutaneous form of disease (78.8%) (Fig. 14.2).
Fig. 14.2
Type frequency of disease: Four cases (7.7%) had the cutaneous form, only 7 cases had the mucosal type (13.5), and 41 cases had the mucocutaneous form of disease (78.8%) (Source: Pooya Khan Mohammad Beigi)
The average time until remission was 7.7 ± 2.9 days for the cutaneous type, and the average Pemphigus Disease Area Index (PDAI) of these patients was 19.5 ± 16.3. In the mucosal type, the remission time was 10 ± 1 days and the average PDAI was 8.3 ± 2.8. For the mucocutaneous form of the disease, the average remission time was 8.9 ± 27 and 10.8 ± 4 days for cutaneous and mucosal types of lesions, respectively.
In the mucocutaneous form of disease, the average PDAI was 17.7 ± 8.3 and 9.7 ± 8.6 for cutaneous and mucosal types of lesions, respectively.
The average of desmoglein (DSG) 3 and DSG1 in the three forms of disease shows no statistically significant differences (Table 14.1).
Table 14.1
Lab and clinical factors
Cutaneous phenotype | Mucosal phenotype | Mucocutanous phenotype | P-value | |
---|---|---|---|---|
Age | 38 ± 9 | 25.5 ± 7.1 | 25.5 ± 7.1 | 0.4 |
Anti-Dsg1 | 297 ± 259 | 14.9 ± 22.1 | 294 ± 320 | 0.04 |
Anti-Dsg3 | 231.2 ± 264.1 | 704 ± 597 | 675 ± 455 | 0.1 |
Cutaneous PDAI | 19.5 ± 6.3 | – – – | 17.7 ± 8.3 | 0.7 |
Mucous PDAI | – – – | 8.4 ± 2.6 | 9.7 ± 8.6 | 0.6 |
For three of the four patients (75%) with the cutaneous form of the disease, remission occurred in less than 10 days, and for one out of the four patients (25%), remission took more than 10 days to occur (Table 14.2).
Table 14.2
Clinical and lab factors of patients in the cutaneous form of disease
Remission in 10 days or less | Remission in more than 10 days | P-value | |
---|---|---|---|
Age
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