Quality of life in patients with oral pemphigus vulgaris.

Results from a single center

in Mexico.
Authors.
Andrs Tirado-Snchez, *,**
Alexandro Bonifaz, *
Berenice Macas-Jimnez, *

Affiliation.
* Department of Dermatology, Hospital General de Mxico.
** Department of Dermatology, Hospital General de Zona 29, Instituto Mexicano
del Seguro Social.

Corresponding author.
Andrs Tirado Snchez, Servicio de Dermatologa, Hospital General de Mxico,
Dr. Balmis 148, Col. Doctores Deleg. Cuauhtemoc, Mxico, D.F, Email:
andrestiradosanchez@hotmail.com

Abstract.
Background. Pemphigus vulgaris is a rare and disabling bullous disease which
leads to considerable morbidity or even death. The importance of quality of life
seems to be relevant in pemphigus outcome; however relevant published studies
are quite few.
Objective. Use a validated questionnaire for evaluating quality of life in patients
with pemphigus vulgaris before and after therapy.
Methods. Forty patients seen in the department of Dermatology with pemphigus
vulgaris were included in the study. Patients completed the questionnaire after
undergoing treatment to assess their quality of life.
Results. The overall score for all the patients was 84.55 18.43. The most
compromised elements of the questionnaire before treatment was pain and
functional limitation and social and emotional status. One month after treatment the
most compromised elements were pain and functional limitation and medication
and treatment.
Conclusion. Even after treatment, oral pemphigus vulgaris negatively affect
patients quality of life. The use of validated questionnaires may allow physicians to
more effectively care for their patients with this condition.

Key words. Pemphigus vulgaris; quality of life; questionnaires.

Introduction.
Pemphigus vulgaris (PV) is a rare and disabling bullous disease of the skin and
mucous membranes. It presents with painful and non-healing oral erosions often
followed by cutaneous, flaccid blisters, resulting in easy rupture and painful
superficial erosions. This unpleasant sensation leads to difficulty in eating and a
considerable morbidity with physical, social, and psychological consequences or
even death.1 Despite the anticipated and substantial impact that PV imposes on
patients quality of life (QoL), relevant published studies are quite few.

2-7

Moreover, systemic glucocorticoids and adjuvant drugs, the mainstay of therapy in

PV, are frequently associated with additional negative impact on QoL.

The aim of our study was to measure the QoL of oral PV patients using a validated
tool, and if therapy impacts on QoL.
Methods.
After approval from the institutional ethics committee, eligible patients who signed
written informed consent were enrolled from January 2013 to December 2014. The
study sample consisted of 40 patients receiving treatment in the Department of
Dermatology. All patients had a clinical, histopathological and immunological
diagnosis of oral pemphigus vulgaris.
New or consecutive patients, older than 18 years, with diagnosis of oral PV were
eligible for the study. Individuals with chronic oral mucosal disease different from

PV; who did not undergo any treatment; patients who found it difficult to understand
the questions and mentally disabled patients were excluded.
Patients complete the Chronic Oral Mucosal Diseases Questionnaire (COMDQ),
an oral health-related QOL instrument containing 26 items (maximum possible
score, 104); 9 each patient complete the questionnaire before treatment starts
(newly diagnosed or after relapse) and one month later. The items are grouped
according to clinical judgment into 4 domains: pain and functional limitation
(maximum possible score, 36), medication and treatment (maximum possible
score, 24), social and emotional status (maximum possible score, 28), and patient
support (maximum possible score, 16). For each questionnaire, patients answered
by using a Likert-type response scale coded. Responses were added to give a total
potential score of 104. Raw scores were then converted to percentages. A score of
0% to 25% was considered an excellent QoL; 26% to 50%, a good QoL; 51% to
75%, a moderate QoL; and 76% to 100%, a poor QoL. The data were entered and
proofread by 2 independent researchers (Dr. Tirado and Dra. Macias).
Statistical analysis was performed using the statistical software SPSS version 17.0,
Chicago, IL. Differences were defined as statistically significant at P< .05.
Results.
Patient demographics are displayed in Table 1. Forty patients (28 female, 12 male)
with an average of 42.8 12.6 years were enrolled. The average duration of the
disease was 4.7 months. Burning sensation was noted in 47.8% and pain in 94.5%

of the patients. The severity of oral cavity lesions were as follows: mild severity, 5
patients (12.5%), moderate, 26 patients (65%) and severe 9 patients (22.5%).
The overall COMDQ score for all the patients was 84.55 18.43. We observed
clear differences between the scores according to the severity of PV. (Fig 1) The
most compromised elements of COMDQ before treatment were pain and functional
limitation (26.3 12.37), and social and emotional status (25.7 9.46). One month
after treatment the most compromised elements were pain and functional limitation
(23.47 8.55) and medication and treatment (18.7 6.74). (Table 1) We observed
a significant correlation between pain and functional limitation and social and
emotional status before treatment (r= 0.674, P= .001), and significant correlation
between pain and functional limitation and medication and treatment one month
after treatment (r= 0.589, P= .001). The other elements correlated between them,
indicating that all four variables affected the patients QoL before and after
treatment. Improvement in any or all of these variables could positively affect the
QoL.
Overall quality of life was not affected by age and sex, however, duration of the
disease correlated inversely with QoL and disease severity score significantly
correlated with QoL (r= 0.049, P= .001 and rs= 0.684, P= .001, respectively).
Discussion.
We found that QoL was significantly affected in patients with PV. This asseveration
is explained on the basis of pain and functional limitation, medication and treatment
and social and emotional status of the patient.

With increasing disease severity, we observed a worse QoL that was attributed
mainly to the worsening of pain and functional limitation, characteristic in PV. After
one month of treatment, the worse QoL was attributable to disease severity, and
also to the treatment side effects. Social and emotional status is an important
factor that affects QoL independent of disease severity and disability related and
treatment; moreover, proper education about PV, patient counseling, and
assurance about the success of available treatment modalities must be included in
the management of PV as recommended on literature consulted.

10,11

Ghodsi et al 3 studied QoL in 61 patients with PV using a version of the

Dermatology Life Quality Index. They observed a negative correlation between the
index and duration of the disease and concluded that PV is responsible for great
alteration of QoL, especially on its severe stage.
In our study, we found that pain and functional limitation secondary to disease is an
important element that impact on QoL with or without treatment, moreover, it
should be properly evaluated and treated when possible to help improve the
patients QoL.
Rajan et al 12 found that questionnaires, like COMDQ, provide a detailed picture of
the impact of oral diseases, including PV, on patients, which add information that
may be useful in clinical practice. This questionnaire may help give a greater focus
to the limited time available at follow-up appointments. Furthermore, the COMDQ
may allow the patient to assist in the evaluation and assessment of treatment
effectiveness and it could supply valuable information regarding the patients

perspective on QoL, which helps the clinician to modify the strategy for managing
PV.
Limitations of our study include that long-term use of therapy was not evaluated;
there was a lack of uniformity in treatment schemes, there was not a control group
and finally, psychiatric morbidity was not evaluated, which is known to impact on
QoL, however, none of the patients had previous reports of psychiatric diseases or
related treatment.
The clinical evaluation of QoL, may give information about the cause, can help to
chose a specific treatment, and can also provide clues about the prognosis,
although it cant reflect the resulting level of impairment. These measurements
must be simple and practical enough for the clinician and patient to use and
interpret. We found the test reliable, simple to use, and sensitive to clinical
parameters and treatment modalities.
Finally, the initial and consecutive evaluations and management of PV must take
into account the impact of QoL on patient outcome.
References.
1. Saha M, Bhogal B, Black MM, Cooper D, Vaughan RW, Groves RW.
Prognostic factors in pemphigus vulgaris and pemphigus foliaceus. Br J
Dermatol 2014; 170(1): 116-22.
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Messer G, Rzany Fr Die Deutschebsd-Studiengruppe B. Significant
decrease in quality of life in patients with pemphigus vulgaris. Results from

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Ges 2005; 3(6): 431-5.
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Quality of life and psychological status of patients with pemphigus vulgaris
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Tables.
Table 1. Patients characteristics.
Variable.

Oral PV (n= 40) Before

treatment.

Age (years) SD

Oral PV (n=40) 1-month

after treatment.
42.8 12.6

Gender (Male/Female) %
Disease severity (%)
Mild
Moderate
Severe
COMDQ SD
Elements of COMDQ
Pain and functional

12/28 (30/70)
5 (12.5)
26 (65)
9 (22.5)
84.55 18.43

8 (20)
28 (70)
4 (10)
72.45 12.76 *

26.3 12.37

23.47 8.55 *

limitation
Medication and treatment 18.36 16.43
18.7 6.74
Social and emotional
25.7 9.46
19.2 6.78 *
Patient support
13.12 10.32 83.48
8.56 7.34 *
COMDQ= Chronic Oral Mucosal Diseases Questionnaire. SD= Standard deviation.
* Statistically significant differences. P-value= .05

Figures.
Fig 1. Disease severity and quality of life according to COMDQ.