A retrospective study of patients with pemphigus during a five-year period, 1988-1992, in the Dr. Cipto Mangunkusumo Hospital, Jakarta

162

Citrarasnù and Djuanda _{Med J Univ Indon}

A

Retrospective Study

of Patients

with

Pemphigus

during

a Five-year Period,

1988-1992,in the

Dr.

Cipto

Mangunkusumo

Hospital,

Jakarta

A.A.A.Made Citrarasmi, Adhi Djuanda

Abstrak

Telah dilakukan penelitian refiospektif para penderita penrfigus selanw 5 tahun (1988 - 1992 _). Diagnosis berdasarkan anantnesis,

ganbaran klinis, petneriksaan histopatologilç dan ùnunoJluoresensi. Selama itu terdapat 26 kasus pemfgus terdiri atas 19 wanita dan 7 pria, berunw antara 14 sanpai 78 tahun. Jenis penrfigus ialah pentfgus vulgaris 10 kasus, pentfigusfoliaseus t2 kasus, dan pentfigus eritenatosus 4 kasus. Inna penyakit antara

I

bulan hingga 2 tahun, lama rawat-inap aniara 5 hari sanpai 5 bulan. Pengobatan yang utana ialah kortikosteroid, dosis awal deksa,netason antara 15 santpai 20 tng dan prednkon antara 3O sampai 8O tng. Terdapat 2 lcasus petnfigus vulgaris dan 3 kasus _{penfigus foliaseus yang meninggal, yaitu 4} knsus disebabkan oleh bronknpneuurcnia dan

I

kasus larena septikania.

Abstract

A retrospective study of penphigus cases during afive-year period, _from 1988 to 1992, was perforned. The diagnosis was based on history, clinical picture, histopathologicalfinding, and innunofluorescence exatnination. During afive-year period there were 26 cases of pemphigus, cotrsisting of 19 _{fenales and} 7 nnles, their ages variedfron 14 to 78 years. The _following types were recognized :

penryhigus vulgaris in IO cases, pemphigus _{foliaceus in} 12 cases, and pemphigus erythenatosus in 4 cases. The duration of illness was between

I

nonth to 2 years, the average hospitalization ranging _from 5 days to 5 nonths. The tnain therapy was corticosteroids with an initial dose of detamethasone between 15 to 20 nry and prednisone with naxinnl dose 80 nry daily. Two cases of penphigus vulgaris and 3 cases ofpemphigusfoliaceous was died, due to bronchopneunronia in 4 cases and septicaenia in

I

case.

Keywords : Penphigus, Itrcidence

INTRODUCTION

Pemphigus refers

to a

group

of

autoimmune intra-epidermal blistering diseases

of

the skin and mucous

membranes,l and is not related to sex.2 Jews are pro-bably more frequently affected. Pemphigus usually affects people between

11"

1g"

:f

.39 - 601ears, but

it can also occur in childhood and older age.'

Based primarily on the level of blister formation,

the pemphigus group^may be subdivided into superfi-cial and deep forms.'

Typo

of

pemphigus are pem-phigus

vulgaris,

pemphigus foliaceus, pemphigus

erythematosus, and pemphigus vegetans.

The major histologic feature

of all

variants is acantholysis, the disruption of normal cell-to-cell

ad-hesion, which leads

to

intraepidermal blister forma-tion.l

Most patients with pemphigus demonstrate IgG autoantibodies directed against an antigen located on the surface of keratinocytes.'

Although the stimulus for autoantibody

produc-tion

is

.,nk

ro*n, several

mechanisms have been

proposed to explain the pathogenesis of acantholysis.

First

pemphigus

antibodies

induce

acantholysis through

local

stimulation

of the

plasminogen-plas-min system, second pemphigus antibodies fix comple-ment and thereby alter

cell

membrane integrity to produce acantholysis. I

The

first choice

for

treatment

is

corticoste-roids,l'3'4'5 other

tr

suppressive

drugs, gold

therapy,

Before the

usage of corticosteroids the rate of death was 50%.6

Death occurs in the acute disease after a few months,

or

after several years

of

intermittent course, due to

Vol 3, No 3, July - Septenùer 1994

secondary complications such as septicemia, broncho-pneumonia, electrolyte imbalance, and cachexia.2'3'6

Reporting cases

of

pemphigus

is

important to

know the incidence rate, the types of pemphigus, the

result of treatment, its mortality rate, and the cause

of

death.

MATERIAL

AND

METHOD

The records of all patients with pemphigus admitted to

our department during a 5 year-period, from January

l,

1988

to

December 31, 1992

were

collected and studied.

RESULT

A retrospective study of pemphigus patients who were

hospitalized in the Dr Cipto Mangunkusumo Hospital, during a 5- year period, 1988-1992, is shown in Table

l.

There were 26 cases of pemphigus, consisting

of

19 females and 7 males (Table 2). The ages varied from

14

to

78

years

(Table 3). The pemphigus vulgaris patients varied in age from 16 to 69 years, pemphigus

foliaceus

from

14

to

78 years, whereas pemphigus erythemato-sus from 30 to 75 years (Table 1).

The duration of disease ranged from

I

month to 3 years, Patients were hospitalized between 5 days to 5 months.

The types

of

pemphigus consisfed of pemphigus vulgaris

in

l0

cases, pemphigus foliaceus

in

12 cases,

and pemphigus erythematosus in 4 cases (Table 1).

The subjective sign

of

pemphigus vulgaris was itching in 3 cases (II,57o). The general condition was

good

in

5 cases (I9.2%), moderate

in

1 case (3.87o),

poor in 3 cases (11.5%), and apathy

in

I

case (3.8%).

Pemphigus foliaceus with good general condition was seen

in

2 cases (7.7%), moderate

in

3 case (11.5%),

and poor in 7 cases (26.9%). Good general condition in pemphigus erythematosus patients was observed in 2 cases (7.7%), mderate

in

I

case (3.87o), and poor

in

1 case (3.87o) (Table 4).

Among 26 cases

of

pemphigus, 26 had

accom-panying diseases, such as pulmonary tuberculosis in 5 cases, pleuropneumonia in 2 cases, bronchitis in 1 case,

spontaneus pneumothorax in 1 case, heart arrythmia in 2 cases, bronchiectasis

in

2 cases, hypertension

in

3 cases, heart

failure

with

sinus tachycardia in 2 cases,

angina

pectoris, insomnia, myringitis and external

otitis,

thrombocytopenia without

DIC,

hematemesis and melena due

to

erosive gastritis, thoraco - lumbal

osteoporosis, pelvis and first lumbar compresion

frac-ture, renal insufficiency due to acute

glomerulonephri-tis, normochromic normocytic anemia, gangrene

of

Pentphigus during a Five-year Period,

1988-1992

163

the pulp and posttotal laryngectomia due to squamous cell carcinoma of the pharynx respectively in one case

(Table 5). There was a possibility that a patient also

suffered from more than one disease.

Complications

of

pemphigus were

blepharocon-junctivitis in 3 cases, conjunctivitis in 5 cases, bron-chopneumonia in 4 cases, acute pharyngitis and

septi-cemia in 2 cases, rhinopharyngitis and foetor ex ore in

1 case (Table 1).

Among the 26 cases, a histopathologic

examina-tion was performed

in

2l

cases. The result were as

follows

:

8 cases confirmed the diagnosis

of

pem-phigus vulgaris, 9 pemphigus foliaceus, and 4 pem-phigus erythematosus. Histopathologic examination was not performed in the remaining 5 cases since they

had received corticosteroids prior to hospitalization.

Direct

immunofluorescence

which

detects im-munoglobulins (mainly

IgG)

and complement com-ponents such as C3 within the epidermal tissue of the blister and its margin, was performed

in

17 patients.

Only

4

cases were

in

conformity

with

pemphigus

(Table 1).

In

9

cases the examination was not

per-formed.

All

patients were treated

with

corticosteroids

only,9 patients withdexamethasone, while 17 patients with prednisone. The

initial

dose of dexamethasone was 15

to

20 mg/day, whereas prednisone 30

to

80

mg/day. After clinical healing the dose was tapered off.

The side effects I candidosis, vulvovaginitis, fluid retention, and Cushing syndrorne respectively in one

case (Table

l).

The outcome

of

the patients was as follows: 5

cases

died (21.7%), consisting

of

2

cases

of

pem-phigus vulgaris and 3 cases cases of pemphigus folia-ceus. The cause of death was bronchopneumonia in 4

cases and septicemia

in

1 case (Table 1).

DISCUSSION

Pemphigus is a rare disease, Jews are probably more frequently affected,2

it

is not related to sex.2'l-In this

study females were more

frequently

affected than

males (80,8% of

all

the cases). The incidence varied

from 0.5 to 3.2 cases per 100.000 population per year. The most frequent type of pemphigus was pemphigus

vulgaris, followed by pemphigus foliaceus and pem-phigus erythematosus.''''' While

in

this study

pem-phigus foliaceus was the most frequent type followed

by pemphigus vulgaris and pemphigus erythematosus,

whereas

no

case

of

pemphigus vegetans was en-countered.

also

affect

all

ages, including

chil

gus vulgaris is gen^erally seen in

164

Citrarasnù and Djuanda

foliaceus in the fourth or

fifth

decade3

or

30-60 years

old.2 In

this

study

a

different finding was noticed, pemphigus vulgaris occurred

in

16 - 69 years of age,

pemphigus foliaceus

in

14-78 years, whereas pem-phigus erythematosus in 30-75 years.

Subjective itching

is

uncommon

in

pemphigus

vulearis. oainful erosions was the dominant clinical

f""iur".i'{7

Itching was dominant

in

only

3

of

our

cases.

Duration of the disease in this study ranged from

1 month to 3 years. Nine cases were hospitalized more than once. This showed that pemphigus is a chronic disease as mentioned in the literature.

The initial disease in pemphigus vulgaris is in the

mouth.l'3 Among our cases foetor ex

ore

was only

found

in

I

case (3.8%) (Table 1).

The

lesions

of

pemphigus foliaceus often

de-velop

initially

on the face, scalp, chest, or back and may spread to involve large areas of the skin,

Present-ing

as generalized exfoliative

dermatitis,

and oral lesions àre not frequently present.3 Oral lesion as

su-perficial erosive stomatitis was only found in 1 case in

our study.

The eyes can also be affected as pyogenic

con-junctivitis.I'' In thir

study 3 cases with

blepharocon-junctivitis and 5 cases with conjunctivitis were found.

Other mucous membranes, including oropharyngeal.,

pharyngeal, laryngeal, and nasal can be affected,r'o

which was only found in 1 case in this study.

The

first

choice

of

treatment

in

pemphigus is corticosteroids as was done

in all of

our cases. The

dose of prednisone depends on the grade of the disease.

In severe cases the suggested dose is 60-150 mg por day, or 3 mg/body weight.a

If

a very high dose

will

be

given we prefer dexamethasone injection intramus-cularly or intravenously instead ofprednisone because

it is more practical. For either pemphigus foliaceus or

pemphigus erythematosus, the dose was not as high as

for pemphigus vulgaris, only 60 mg/day prednisone or

u.roih".

"o.ticosteroid

with

an "quiuui"nt dose.a's In

our cases, 10 cases were treated with dexamethasone,

with an initial dose ranging from 15 to 20 mg, 16 cases

were treated with prednisone varying from 30 to 80 mg daily.

Med J Univ Indon

Besides corticosteroids we gave also antibiotic to

prevent secondary infection and

ACTH

(synacthen depot), which was given every week, with a dose of 2

mg

weekly to prevent atrophy of the adrenal cortex.4

We only used corticosteroids and was not combined

with cytostatics because we were afraid

of

the side effects.

The side effects of corticosteroids are depression

of the immunity and prone to infection. In our cases,

side effects found were herpes zoster, steroid ac-neiform eruption, oral candidosis, vaginal candidosis

each

in one

case, and active pulmonary tuberculosis

in 2

cases.

Another

side effect

was

steroid

diabeticum in

4

cases.

Corticosteroids also cause electrolyte imbalan-ces and sodium ietention, two cases with

fluid

reten-tion

were found

in

this

study.

The

of

pemphigus is septicemia,

bronchop

chexia,2'3's

and

fluid

im-balance.6

rate

is

60-90 %.1'4 This is in accordance

with our

study, 2 cases

of

pemphigus

vulgaris

(8,7%),

and

3 cases

pemphigus foliaseus

(13%) died, i.e. 4 cases due to bronchopneumonia and

I

case due to septicemia,

REFERENCES

l.

Korman N, Cleveland OH, Pemphigus. Arch Dermatol 1988; l8 (6): 1219-83.

2.

Braun Falco, Plewig G, Wolft HH, Winkelmann RK. Der-matology. 3rd ed. Berlin : Springer-Verlag, 1991: 467-501.

3.

Iordon RE. Pemphigus. In : FitzpatrickTB, Eisen AZ, Wolff

K, Freedberg IM, Austen KF. Dermatology in general medecine, 3rd. New York : Mc Graw Hill, 1987 : 571 - 8.

4.

Djuanda A. Pemphigus. Majalah Kedokteran Indonesia 1973; tUt2 t 2O4-7.

5.

Djuanda A. Dermatosis eritroskuamosa. In : Djuanda A, ed, Ilmu Penyakit Kulit dan Kelamin, lst ed. Iakarta : Medical

University oflndonesia, 1987 : 153 - 63.

6,

Provost Tï,Zrine

II.

Bullous Disease. In : Moschella SL,

Hurley

HI.

Dermatology, 2nd ed. Philadelphia

:

WB Saunders, 1975 : 557 -66.

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Vol 3, No 3, July - September 1994

Table 2. Sex distribution of pemphigus cases

Pernphigus during a Five-year Period, 1988-1992 169

Type of pemphigus

Sex Total Vo

Pemphigus vulgaris

Pemphigus foliaceus

Pemphigus erythematosus Female

Male

10

2

19

7

73,1

26,9

Table 3. Age distribution of pemphigus cases

Type of pemphigus

Age Total 7o

Pemphigus

vulgaris

Pemphigus

Pemphigus

foliaceus

erythematosus

1

3,8

3

Ll,s

5

t9,2

|

7,6

4

L5,4

5

L9,2

3

15,4

L

7,6

;

2

1

2

1

1

I

2

I

2

3

1 1

-14

L5 -22

23 -30

31 -38

39-46

47 -54

55-62

63 -70

>7L

Table 4. General condition distribution of pemphigus cases

General

condition

Type of pemphigus

Total Vo

Pemphigus

vulgaris

Pemphigus

Pemphigus

foliaceus

erythematosus

Good

Moderate

Poor

Apathy

2

3

10 5

38,5

t9.2

38,5

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