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2007 The International Society of Dermatology International Journal of Dermatology 2007, 46, 923926
923
Abstract
Background Pemphigus is a rare, life-threatening, acquired autoimmune bullous dermatosis.
The prognosis of pemphigus foliaceus (PF) is usually regarded as more favourable than that of
pemphigus vulgaris (PV). Our study aims to compare the clinical course of PV and PF in
37 patients.
Patients and Methods We conducted a retrospective study over a period of eight years
(19942001). The patients were referred during this period and were followed until December
2003. PF and PV were included based on clinical, histological and immunopathological criteria.
Results
In our study there was no significant difference between PF group and PV group
concerning; age, sex, duration of the disease, presence of disseminated lesions, treatment,
healing time, remission, relapse, complications, death and follows up duration. The survival graph
showed no difference between the two groups for the first two relapses. There was a tendency
to significance concerning an additional treatment and relapses frequency in the PF group.
Conclusions
Few studies in the literature were interested in the evolution of the two forms of
pemphigus. They showed that the two populations share the same clinical course; nevertheless
they revealed the frequency of partial remission, failed treatment, relapses, necessity of high
dose of corticosteroids, and difficulties of discontinuing treatment in PF. Our study, suggests that
PF and PV may share the same clinical course.
BlackwellPublishingLtdOxford,UKIJDInternationalJournalofDermatology1365-4632BlackwellPublishingLtd,200645
Report
Pemphigusvulgarisandpemphigusfoliaceus
Zaraa
etal.Report
Pemphigus vulgaris and pemphigus foliaceus:
similar prognosis?
I. Zaraa, MD
, M. Mokni, MD
, M. Hsairi, MD
, S. Boubaker, MD
, M. Sellami, MD
,
M. Zitouni, MD
, S. Makni, MD
, and A. Ben Osman Dhahri, MD
From the Department of Dermatology, La
Rabta Hospital, Jabbari, Department of
Preventive Medicine and Public Health, The
National Public Health Institute, Department
of Anatomopathology, Pasteur Institute, and
Department of Immunology, La Rabta
Hospital, Jabbari, Tunis, Tunisia
Correspondence
Mourad Mokni, MD
Department of Dermatology
La Rabta Hospital
Jabbari
Bab Saadoun
1007 Tunis
Tunisia
E-mail: [email protected]
Introduction
Pemphigus is a rare, life-threatening, acquired autoimmune
bullous dermatosis. The prognosis of pemphigus foliaceus
(PF) is usually regarded as more favorable than that of
pemphigus vulgaris (PV); however, the prognosis of both
types of pemphigus has improved considerably since the
introduction of corticosteroids. We compared the clinical
course of PV and PF in 37 patients.
Patients and Methods
Our retrospective study was conducted over a period of 8 years
(19942001) at the Department of Dermatology, Rabta Hospital,
Tunis, Tunisia. The patients were referred during this period and
were followed until December 2003. Inclusion in the study was
based on clinical, histologic, and direct immunofluorescence
(DIF) criteria. Immunoblot studies for desmoglein 1 (Dg1) and
desmoglein 3 (Dg3) were performed in eight PF and nine PV
patients.
PF was defined clinically by the presence of erythematous,
squamous plaques involving seborrheic areas of the face and
trunk, and histologically by acantholytic subcorneal separation.
Immunoblotting demonstrated anti-Dg1 antibodies only in seven of
the eight PF patients. One patient, considered to have PF on
clinical and histologic grounds, had both anti-Dg1 and anti-Dg3
antibodies. In contrast, PV was defined clinically by cutaneous and/
or mucosal blisters, and histologically by suprabasilar intraepidermal
separation. DIF showed intraepidermal immunoglobulin G (IgG)
deposition.
All 22 PV and 15 PF patients were treated initially with
systemic corticosteroids. Results were analyzed using EPI INFO
software version 6.04 and Stata software version 6. To compare
the rates of response to treatment, we used the chi-squaredtest with the Fisher exact test when necessary. We applied a
nonparametric test, the KruskalWallis test, to compare the
quantitative variables in the two groups. In addition, PV and PF
groups were compared for the first and second relapse by the log rank
test. The significance basis was set at 0.05.
Results
The main features of our PV and PF patients are summarized
in Table 1. There was no significant difference between the
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International Journal of Dermatology
2007, 46
, 923926 2007 The International Society of Dermatology
924 Report
Pemphigus vulgaris and pemphigus foliaceus
Zaraa
et al.
two groups in terms of age (49
16 vs. 41
16 years), sex
(female 73% vs. 87% male), average duration of disease before
treatment (12
23 vs. 9
9 months), or presence of dis-
seminated lesions on presentation (59% vs. 80%). The first
lesion site was more frequently cutaneous in the PF group (87%vs. 54%, P
< 0.001) and mucosal in the PV group (77% vs. 7%).
Table 2 outlines the treatment of our PV and PF patients.
All were treated initially with systemic corticosteroids. The
initial average doses were similar in both groups (1.3 mg/
kg/day). At the end of the initial treatment period, the PF
group showed a significantly increased need for additional
treatment (azathioprine, dapsone) (33% vs. 9%, P
= 0.09).
There was no difference between the two groups in terms
of the type and dose of maintenance therapy. The 3- and
6-month doses of corticosteroids were also similar: (38
12
vs. 36
14 mg/day and 21
8 vs. 18
9 mg/day, respectively).
No patient required interruption of treatment during the study.
Table 3 summarizes the course and follow-up. The healing
time (1.9
1.7 vs. 1.6
0.7 months), remission (64% vs.
73%), death (19% vs. 13%), complications (73% vs. 87%),
and follow-up duration (32
34 vs. 32
23 months) were
similar in the PV and PF groups.
A higher tendency for relapse was observed in the PF group
(0.5
0.8 vs. 0.3
1.3, P
= 0.09). The survival graph showed
no difference between the two groups for the first two
relapses (Figs 1 and 2).
Discussion
Pemphigus is a blistering autoimmune disease characterized
by the appearance of intraepidermal bullae. Its classification
is based on the clinical aspect, the histologic cleft level, and
the identification of the antigen target of the autoantibody.
PV, whose lesions start at the oral mucous membranes, is
characterized by a suprabasal cleft and the production of anti-
bodies directed against Dg3. PF, commonly observed on the
face and chest, is characterized by subcorneal acantholysis
and the production of antibodies directed against Dg1.Our study compared the clinical course of PF and PV. The
most important finding was that there was no significant
difference in age, sex, duration of disease before treatment,
presence of disseminated lesions on presentation, treatment,
time to healing and remission, and rates of relapse, com-
plications, or mortality between the two groups of pemphigus
patients. Few studies have directly compared the outcomes of
PV vs. PF. On comparing the two types of pemphigus, Dehen
et al
.
1
showed that they shared the same course. Traditionally,
PF was thought to have a more favorable outcome than PV,
Table 1 Pemphigus: clinical and immunologic findings
PF (n= 15) PV (n= 22)
Age (years) 41 16 49 16
Sex (male/female) 2/13 6/16
Duration before treatment (months) 9 9 12 23
Follow-up care (months) 32 23 32 34
Site of the first lesion
Cutaneous/mucosal 13/1 5/17
Cutaneous lesions on presentation
Trunk 15 17
Face 7 7
Scalp 12 9
Limbs 12 12
Disseminated cutaneous lesions (%) 80 59
Mucosal lesions on presentation (%) 20 86
Immunoblot results
Dg1 positive 8*/8 4/9
Dg3 positive 1*/8 9/9
IF positive 3 1
Dg, desmoglein; IF, immunofluorescence; PF, pemphigus
foliaceus; PV, pemphigus vulgaris.
*In one patient with PF: Dg1, 200; Dg3, 223.
Table 2 Pemphigus: treatment
PF (n= 15) PV (n= 22) P
Initial prednisone dose (mg/kg/day) 1.3 0.2 1.3 0.1 NS
Prednisone dose at 3 months (mg/day) 36 14 38 11 NS
Prednisone dose at 6 months (mg/day) 18 9 21 8 NS
Additional treatment (%) 33 9 NS
Prednisone with:
Azathioprine 3 2
Dapsone 2 0
NS, not significant.
Table 3 Pemphigus: clinical course
PF (n= 15) PV (n= 22)
Relapse
Frequency (%) 53 32
Average number (extremes) 0.5 0.8 (0 4) 0.3 1.3 (06)
Mean t ime/treatment (months) 13 10 13 11
Mean time/first remission (months) 11 10 12 11
Remission
Frequency (%) 73 64
Duration (months) 1.6 0.7 1.9 1.7
Iatrogenic complications (%) 87 73
Infection (septicemia) 10 (4) 15 (4)
Diabetes 4 5
Psychiatric problems 2 0
Corticosteroid myopathy 0 1
High blood pressure 0 2
Aseptic osteonecrosis 1 0
Deaths (%) 13 19
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2007 The International Society of Dermatology International Journal of Dermatology
2007, 46
, 923926
925
Zaraa
et al. Pemphigus vulgaris and pemphigus foliaceus
Report
although some studies have demonstrated frequent partial
remission, treatment failure, relapse, the need for high-dose
corticosteroids, and treatment dependence.
24
Corticosteroid
use has narrowed the difference in morbidity and mortality
between PV and PF.
1
The mortality rates in our cases werein line with literature data for PV (19%) and PF (13%).
1,5
Prior to the use of corticosteroids, significant mortality
was associated with pemphigus (4379%), with most deaths
occurring during the first 23 months. Today, mortality is
mainly associated with treatment complications.
1,6
In our study, systemic corticosteroids were not discontinued
in any patient. The absence of prospective studies and
well-controlled trials has contributed to the difficulty in
determining optimal initial and maintenance regimens.
Fernandes and Perez
5
indicated that prednisone, at doses of
up to 100 mg/day, provided good initial control of pemphigus
and did not increase mortality. In contrast, prednisone doses
of 120 mg or higher were associated with higher morbidity.
Herbst and Bystryn
7
suggested that complete and lasting
remissions could be induced in most patients, and systemictherapy could be safely discontinued without a flare in disease
activity. In conclusion, our study suggests that PF and PV
share the same clinical course.
References
1 Dehen L, Cricks B, Grossin M, et al.
Comparative study of
the course and prognosis of pemphigus vulgaris and
pemphigus erythematosus. Ann Dermatol Venereol
1993;
120
: 874878.
Figure 1 Survival graph: first recurrence
Figure 2 Survival graph: second
recurrence
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2007, 46
, 923926 2007 The International Society of Dermatology
926 Report
Pemphigus vulgaris and pemphigus foliaceus
Zaraa
et al.
2 Rayan JG. Pemphigus. A 20 years survey of experience with
70 cases. Arch Dermatol
1971; 104
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3 Perry HO. Pemphigus foliaceus. Arch Dermatol
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4 Perry HO, Brunsting LA. Pemphigus foliaceus. Further
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5 Fernandes NC, Perez MT. Treatment of pemphigus vulgaris
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6 Rosenberg FR, Sanders S, Nelson CT. Pemphigus.
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Arch Dermatol
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7 Herbst A, Bystryn JC. Patterns of remission in pemphigus
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