Research Results




(1)
Misdiagnosis Association & Society, Seattle, WA, USA

 



Keywords
Pemphigus vulgarisDesmogleinPemphigus Disease Area Index (PDAI)Mucosal remissionCutaneous remission


Fifty-two pemphigus vulgaris patients were investigated in the author’s previously unpublished study.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).

A430108_1_En_14_Fig1_HTML.gif


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).

A430108_1_En_14_Fig2_HTML.gif


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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Apr 13, 2018 | Posted by in Dermatology | Comments Off on Research Results

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