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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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International Journal of Dermatology

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

: 1420.

3 Perry HO. Pemphigus foliaceus. Arch Dermatol

1961; 83

:

106125.

4 Perry HO, Brunsting LA. Pemphigus foliaceus. Further

observations. Arch Dermatol

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5 Fernandes NC, Perez MT. Treatment of pemphigus vulgaris

and pemphigus foliaceus: experience with 71 patients over 20

year period. Rev Inst Med Trop Sao Paulo

2001; 43

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6 Rosenberg FR, Sanders S, Nelson CT. Pemphigus.

A 20 year review of 107 patients treated with corticosteroids.

Arch Dermatol

1976; 112

: 962970.

7 Herbst A, Bystryn JC. Patterns of remission in pemphigus

vulgaris.J Am Acad Dermatol

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