UIP HEALTH MED.

, 2016, 1(1)
doi: http://dx.doi.org/10.7454/uiphm.v1i0.21

Oral Pemphigus Vulgaris: The Importance of Patient’s Adherence

Wahyuning Ratnawidya1, Anandina Irmagita Soegyanto2*

1.
Oral Medicine Residency Program, Faculty of Dentistry, Universitas Indonesia, Jakarta 10430, Indonesia
2.
Oral Medicine Department, Faculty of Dentistry, Universitas Indonesia, Jakarta 10430, Indonesia

*E-mail: a.irmagita@gmail.com

Abstract
Background: Pemphigus vulgaris (PV) is an autoimmune mucocutaneus disease characterized by intraepithelial bulla
formation involving the oral mucosa. The treatment requires long-term corticosteroid at variable doses according to the
severity of the lesion.
Case report: A 41-years-old male was referred from the Dermatology Department with oral manifestations of PV. He
was treated with systemic methylprednisolone. Oral examination showed severe intra oral PV lesion so that an
additional of dexamethasone mouth rinse was prescribed. After 4 weeks of treatment, oral and systemic lesions were
relieved. At that time the patient believed he has recovered and decided to stop the medication by himself. Several
months later, his skin and oral lesion relapsed with more severe clinical condition than the previous one and he was re-
treated with corticosteroid. The severity of PV was suspected by the lack of adherence in medication.
Conclusion: Discontinuation of corticosteroids without a rational reason might influence the recurrence and severity of
PV. Good adherence toward corticosteroid therapy is an important factor in PV treatment.
Keywords: corticosteroid, patient’s adherence, pemphigus vulgaris

Introduction Case report

Pemphigus vulgaris (PV) is an autoimmune mucocutaneus
disease characterized with flaccid blister and erosion of
the skin and mucous membranes.1The etiology is still
unknown but strongly associated with HLA class II genes.
The worldwide incidence is 0.1–0.5 per 100,000.1 PV can
occur in all racial and ethnic groups with the highest
incidence in Ashkenazi Jews during 40–60 years old.2
Histological examination of PV will show acantholisys.
The main therapy of PV is long term corticosteroid which
combined with steroid sparing agent.1 The main goals the
treatment is to reduce the symptoms, maintain organ
function due to long term corticosteroid or
immunosuppressant agent and controls the autoimmune
process by induce long period remission and or reduce the
flare up. At the end, the treatment should improve the
patient’s quality of life.
There is a need for patient’s adherence, in order to reach
the successful treatment of long term therapy, that should
be applied also in PV therapy. Adherence is more than
just performed on taking medication but it also requires
patient intervention to be active partner with health
professionals.4 This case report will discuss about the
importance of patient adherence during PV treatment.
A 41 years old male patient was referred to Oral Medicine
clinic of International Hospital in Jakarta from
Dermatoveneorology with diagnose as PV for 7 months
with the biopsy result show suprabasal clefting, row of
tombstone and acantholytic cell. Patient confessed the
presence of oral lesion prior the skin lesion, but he took
self-medication and the lesion healed. But it reappeared
since 3 weeks and got worse, although with lessens pain
and no swallowing complains at the moment. His oral
lesion was also accompanied with blister lesions on his
face, back, arm and leg. He admitted that he had delayed
his follow up to Dermatology clinic for 4 months. The
patient was a high school graduated entrepreneur who
supports the family finance. He had no familial history of
PV and the disease was suspected to be triggered by
psychological stress. The medication from Dermatology
department contains systemic Methylprednisolone (MP),
Azathioprine, Clindamycin, Ranitidine®, with Calcium
and D3 vitamin supplement. His skin lesion was treated
with NaCl 0,9% and salicylic acid.
Extra oral examination showed multiple crusted and
erosion area on face, chin and neck, with soft bulla
presence on his leg. The submandibular lymph nodes were
tender on palpation without any pain. There was multiple
bleeding crusted and desquamation areas on his upper and

39
lower lips. Intra oral examination was revealed multiple
sloughing areas, ulcers and erosion in all mucosal surfaces
(Figure 1).

Oral hygiene was poor with debris, sub and supra gingival
calculus and tooth staining. The oral lesion was diagnosed
to be intraoral manifestation of PV.

The patient management consisted of communication,

information and education (CIE) about oral manifestation
of PV and treatment plan. An oral rinse to be swished and
spit was prescribed that contained dexamethasone with as
much as 6 mg in the morning and 3 mg in the afternoon.
The lip lesions were treated with lip ointment mixture that
Figure 2. Last follow up visitin oral medicine, October
contained antibiotic, corticosteroid, and moisturizer, to be
29th 2015
applied thrice a day. The patient was also instructed to
brush his teeth twice a day with soft toothbrush.
The patient was absent from his scheduled follow up visit
for about 3 months. Then, his PV lesions relapsed with
skin-mouth-eye involvements that required him to be
hospitalized. He complained for pain and limited mouth
opening. On inward visit, clinical examination showed
yellowish-black crusted and bleeding lesions on the lips
and circum oral areas, while there was bulla on the right
hand and erythematous eye with purulence. Intra orally,
there were sloughing and erosion in buccal mucosa and
the tongue (Figure 3).

Figure 1. First visit in oral medicine clinic, October 7th

2015

Figure 3. First inward visit, January 12th 2016

One week later, on the first follow up visit, the patient
experienced new lesion on his fingers and painful oral
mucosal lesion for 3 days. He admitted to take his
medication according the doctors instruction. Extra oral
examination showed black crusts on upper and lower lip.
Intra orally, there were no significant difference from the
first visit. Similar medication to the prior visit was
prescribed to this patient for 2 weeks’ period.
The treatments given for oral lesion were customized
technique for oral hygiene procedures, dexamethasone
solution on the same dose as his first visit, along with lip
ointment and moisturizer. The dermatologist also gave the
same medication as his previous one, although with higher
dose with an addition of eye drops for the eye lesion.

On the next follow-up visit, the patient admitted that there

was neither new oral nor skin lesion, and the pain was
decreased. Clinical examination showed improved oral
and skin lesion. The crusted-on lips were decreased in size
while the ulcerationand erosions seen to be improved in
palate, buccal and labial mucosa. (Figure 2.) The
medication prescribed was still the same with previous
visit which is oral rinse to be swished and spit was
prescribed that contained dexamethasone with as much as
6 mg in the morning and 3 mg in the afternoon. The lip
lesion was continued with lip ointment mixture.

The patient was instructed to have a follow up visit the
next week but he didn’t come. When contacted by phone,
he declared that to be recovered and refused to come for
follow up. The dexamethasone oral rinse had not been
tappered yet and the patient claimed that he continued to
all the prescribed medications as instructed.
Figure 4. Inward follow up visit, January 25th2016
The treatment was continued for another two weeks
without any change on regular inward visit. On the last
inward visit, the patient had no complaint about pain or

40
swallowing. The intra oral lesion of mucosa showed
lessen erosion (Figure 5). Skin lesion showed less erosion
with squama. At that moment, the patient was discharged
from the hospital. For home care, the oral treatment was
still maintained as before with the intensification of oral
hygiene procedure. The dermatologist prescribed the
corticosteroid in tapered dose with supplements but a
change of immunosuppressant agent into Mycophenolate
mofetil. The patient and his family was also reeducated,
then was given oral and written instruction on the use of
medication, oral hygiene maintenance and the importance
of follow up visit. He was planned to have a follow up
visit within a week. But, again, the patient did not come
and this time he could not be recalled.
Figure 5. Last follow up visit, February 9, 2016
Discussion
This case report describes a Pemphigus Vulgaris patient
who had oral and skin involvement. The oral lesion erupts
in the first place but he did not come to dentist at the first
eruption, since he did not feel any discomfort at that time.
The patient finally came to the dentist after had some oral
discomfort and referred from Dermatovenerology
department. In most cases, PV lesion appears initially on
the oral mucosa, and can be located anywhere within the
oral cavity with the most common found in buccal
mucosa1. The occurrence of oral lesion is related to the
circulating auto antibodies against desmosome molecules
of Desmoglein 1 (Dsg1) and Desmoglein 3 (Dsg3) which
directed majoring Dsg3. Oral mucosa prominently express
Dsg3. Auto antibodies circulating induce loss of adhesion
cell in epithelial suprabasal and forming of intraepithelial
vesicles or bulla.2
The patient was treated with systemic corticosteroid for
his skin lesion, while the oral lesion was treated with
topical corticosteroid. Systemic and topical corticosteroids
are the first line therapy of PV.3 The corticosteroids
therapy has important function as anti-inflammatory and
immunosuppressant 5 Although the patient was already
given with systemic corticosteroid therapy, but he was
still given the topical ones since oral lesions often
unaffected by systemic medication. Oral lesion can be
managed with topical corticosteroid rinse or cream.6
Topical treatments of corticosteroid can speed up re-
epithelialization of pemphigus lesions include epidermal
41
growth and decrease disease progression and persistently
decreased desmoglein-reactive antibody titer.7
The treatment of PV patient requires long term use of
immunosuppressant agents. Here in our case, the patient
was treated for almost a year. Without any treatment, PV
lesions can spread more extensive and life-threatening
fatal within 1 to 3 years, because of loss of fluid and
protein or opportunistic infection.2 The wide variety of
therapies can be used but no one can guarantee a
permanent remission and sometimes recurrence may still
happen.6 The patient was also treated with steroid sparing
agents such as azathioprine and mycophenolate mofetil.
These are immunosuppressive non-steroidal drugs steroid
sparing agents, used to reduce adverse effect of long term
corticosteroid without eliminate the beneficial effect of
corticosteroid5. The long-term use of corticosteroid and its
sparing agent can cause various side effects.5,8,9
In this case, the patient frequently stopped his medication
without health professional advice, including his
corticosteroid treatment, every time he felt any
improvement of his condition. When abruptly
discontinued, long term corticosteroid will cause adrenal
insufficiency.5 The patients stopped the medication, and
for several times, happened when the corticosteroid doses
were not tapered down yet. The dose of corticosteroid can
be tapered by 25% if remission is induced and maintained
with healing of the majority of lesion.3
The relapses of PV lesions happened in this case. The
etiology of PV is unknown but strongly associated with
genetic some HLA class II genus.2 Some inducing factors
such as drug intake, viral infections, ultra violet radiation,
nutrition, chemicals, and emotional stress.2 The patient in
our report was never in complete remission, since the
lesion of PV can be declared to have complete remission
without treatment when no new lesions appear without
therapy for two months.1 The relapses of this case might
happen due to the frequent discontinuation of medication
or the presence of inducing factors that had not been
identified yet.
The frequent medical treatment discontinuation showed
the patient’s poor adherence toward PV medication. Poor
adherence may associate with confusion, forgetfulness,
language difficulty and feeling good enough to abstain
from medication10. World Health Organization (WHO)
classifies the barrier for medication adherence into 5
categories. These are barriers in socioeconomic factors,
healthcare organizations, disease related, therapy-related
factors and patient-related factors.4 In this case, the patient
adherence might be related to barrier of patient-related
factors. There is also a possibility of economic factor that
took a role in this case, since the patient admitted himself
as the financial backbone of his family and still had to
work even when he wasn’t recovered yet.
During PV treatment, the patient was given oral and
written medical information and instruction. That was also
to the family during inward treatment period. The content
of information includes life-threatening diseases, the
recurrences, and the purpose treatment using
corticosteroids and long-term side effects that might occur
without medication. The patient was also persuaded to
have good adherence and self-care during the medication
period but the patient failed to perform good adherence
towards dentist instruction. Patient’s health attitude and
literacy suspected to be the main factor of non-adherence
to complete the medication. Medical adherence also can
be influenced by the level of awareness of the health and
socioeconomic status. The ability of patients to bond with
the doctor's information may be influenced by the level of
knowledge and patient’s expectations.11 In adherence,
patient plays active role as partner and collaborate to
dentist about the medical treatment.4
On this case report, non-adherence is strongly suspected
as a factor that worsened the severity and recurrences of
PV. Lack of adherence will have an impact toward the
increased of diseases complication and reduced quality of
life which resulting higher health care cost.12 Non-
adherence increases the risk of morbidity, hospitalization
and mortality with the result on decreasing health. To
increase patients’ adherence, dentist’s communication
skills and good manners also plays important role.13 One
of communication technique that can be applied by dentist
is teach-back method, where the patient or family are
asked to show or do the dentist’s information or
instructions to ensure their acceptance.14 The availability
of good information, communication and support to
patients can increase the engagement and involvement of
patients in medical treatment.13
Conclusions
The long-term use of corticosteroid in Pemphigus
Vulgaris treatment requires a good patient adherence.
There is a need of good communication between the
medical professional and patients/family to gain optimal
adherence in order to achieve the best treatment result.
Acknowledgment
The authors would like to acknowledge drg. Siti Aliyah
SpPM, drg. Indriasti Indah Wardhani SpPM and drg.
42
Endah Ayu Tri Raharjo SpPM, drg Theodorus Hedwin K.
SpPM for assisting the patient’s clinical examination.
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