Introduction
Pemphigus Vulgaris (PV) is a potentially fatal autoimmune mucocutaneous disease (MCD).1 Pemphigus Vulgaris is considered the most common type of pemphigus and comprises approximately (80%) of the reported cases among literature worldwide2 The most common affected sites include cheeks, lips, palate.3 It develops most commonly during the 5th to 7th decade of life4 affecting female more than males.5 The estimated worldwide mortality rate is approaching 5-10%.6
Objectives
To study the Socio - demographic factors and Oral mucosal involvement of PV inpatients at Khartoum Dermatology and Venereal Diseases Teaching Hospital KDVTH.
Materials and Methods
A retrospective - hospital based study was conducted at KDVTH, Sudan from January 2010 to December 2019. From a total of (817) records of MCDs, (678) were included in the survey, of which (321) were selected on basis of the following criteria:
Results
In the survey, (321) records were found reported with PV (47.3%), as the most common MCD.
Most of cases were adults falling between 20-60 years. Patients between 1 and 20 years constituted only (9.7%) of the sample, with only 10% of them over 60 years, as shown in Table 1. Females to male ratio was (2:1).
Among patients, OMLs were reported in 227 (70.7%) case, with only 5 cases reported with OMLs alone, and compared to 222 patients with both OMLs and skin lesions. The majority of OMLs (81.9%) were reported in more than one site, with lips as the most common reported OMLs site (23.5%), as shown in Table 2. As shown in Table 3, the most common recorded OMLs were erosions (42.8%), then bulla (21.6%), followed by Crustations (14.4%).
About One third (32.1%) of cases were associated with chronic systemic diseases. The overall frequency of mortality reported was 35 cases (10.9%), with 29 cases (82.9%) reported with OMLs, compared to only 6 cases (17.1%) without OMLs.
Table 1
Shows age distribution among study population
|
PV |
1 to 10 year |
11 to 20 year |
21 to 30 year |
31 to 40 year |
41 to 50 year |
51 to 60 year |
61 and above |
Total |
|
4 |
27 |
64 |
67 |
67 |
60 |
32 |
321 |
|
|
(n) |
||||||||
|
% |
1.3 |
8.4 |
19.9 |
20.9 |
20.9 |
18.6 |
10.0 |
100 |
Table 2
Shows detailed OMLs site distribution (n, %) among pemphigus vulgaris patients
|
PV |
Cheek |
Tongue |
Lip |
Palate |
Gum |
Others |
Total |
|
(n) |
23 |
59 |
99 |
88 |
18 |
134 |
421 |
|
% |
5.5 |
14.0 |
23.5 |
20.9 |
4.3 |
31.8 |
100 |
Discussion
Few studies have been conducted to describe OMLs involvement and epidemiology of PV in Sudan, with limited research available worldwide as well.
The current study supported evidence from similar reports [Suliman et al. 2013; V,lckova-Laskoska et al. 2007; Seo et al. 2003; Uzun et al. 2006; Zaraa et al. 2011] that PV was the most common reported MCD in this survey (47.3%).7, 8, 9, 10, 11
The most common age groups reported in this survey were (31-40), (41 – 50) years, each with (20.9%) with a mean age of (40.3 ± 12.5) years. It is encouraging to compare these outcomes with that found by Seo et al. 2003; Daneshpazhooh et al. 2009; Shamim et al. 2008.9, 12, 13
Consistent with literature [Lamey et al. 1992; Firth et al. 1991], the current survey indicated that Females were more affected than males with a ratio of (2:1).14, 15
The current survey reported a high frequency of OMLs (70.7%), this is consistent with what was reported by Mignogna et al. 2009 (66%).16
The results of this survey indicated high association between OMLs and skin lesions (69.2%). This outcome can be compared with those obtained by Saraf. 2016 (85%); who reported high association (more than 50%).17
The most obvious finding to emerge from the analysis of OMLs involvement is that more than one site was affected (81.9%), and Lips were the most common reported OMLs site (23.5%), this is in agreement with Kapoor et al. 2013, in which more than half of cases, multiple sites were recorded with OMLs, and with Munhoz et al. 2011, in which Lips were the most common reported OMLs site.6, 18
The current survey indicated that the most common reported OMLs were erosions (42.8%). This outcome is in agreement with Suliman et al. 2013; Saraf. 2016; Shamim et al. 2008; Uzun et al. 2006, Firth et al. 1991; Kapoor S et al. 2013, who suggested erosions as the leading OMLs.7, 17, 13, 10, 15
The current survey claimed that about 1/3 (32.4%) of PV cases were associated with chronic systemic diseases [Significant]. This outcome can be compared conveniently with Uzun et al. 2006 (28.1%); and less conveniently with Seo et al. 2003 (21.1%).10, 9
The most obvious finding to emerge from the analysis is that the frequency of mortality reported in this survey was (10.9%), which is high, but still can be compared with the estimated world wide range (5 - 10%), which is well supported by evidence from Chi et al. 2006; Kulthanan et al. 2011; Abualgasim & Yousif. 2020.5, 19, 20 Among which (82.9%) were associated with OMLs and the peak age group reported was (41 – 50) years by (41.5%), the majority of which were females by (78.9%). This is in agreement with Uzun et al. 2006 in regard of peak age of mortality (50 ±10) years and females predilection.10
Conclusion
Pemphigus Vulgaris was the most frequent MCD (47.3%), well-supported by wide range of literature. Most of the ages of patients fall between 31 and 50 years (41.8%), and females were twice as affected as males. The frequency of OMLs was high (70.7%) (Significant), with high skin lesions association (69.2%). Most of the OMLs were presented at multiple sites (81.9%), and the Lips reported as the most frequent site (23.5%). One third of cases (32.4%), were associated with chronic diseases (Significant). Although, the frequency of mortality with PV in this survey was high (10.9%) (Significant), but still can be compared to the estimated worldwide range 5-10%.
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