LMC journal Vol. 2.indd


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Clinical Study of Pemphigus in Lumbini Medical College
Mikrani JA, Sharma U and Thapa S 

Department of Dermatology and Venereology, Lumbini Medical College, Palpa, Nepal

Corresponding author: Dr. Mikrani JA, Lecturer, Dept. of Dermatology and Venereology, Lumbini Medical College, Palpa, 
Nepal; e-mail: drjameelmikrani@gmail.com

ABSTRACT
Background: Pemphigus is a worldwide disease and varies in its clinical profi le and epidemiology in diff erent 
regions of the world. The disease is rare and few epidemiological data are available. Objec  ve: The purpose 
of this study was to evaluate the epidemiologic, clinical, and therapeu  c features of pemphigus in Lumbini 
Medical College.  Methods: We retrospec  vely inves  gated a total of 70 pa  ents with pemphigus. The 
parameters including age at diagnosis, sex, diagnos  c methods and treatment outcome of the disease. Results: 
The mean age at the onset was 41.3 years. The male-to-female ra  o was 1.18:1. Most of the pa  ents were 
cured at the end of the treatment. Rest were clinically improved. Conclusion: Pemphigus vulgaris (PV) is the 
most common clinical subtype in our centre. The disease  is more frequent in the fi  h decade of life and has 
a male predominance in our region. 

Keywords: Pemphigus, dermatological disease, epidemiology, clinical , therapeu  c

Original Article L M Coll J 2013; 1(2): 83-85

INTRODUCTION
The term pemphigus refers to a group of autoimmune 
blistering diseases of the skin and mucous membranes 
characterized histologically by intraepidermal blister 
and acantholy  c cells and immunopathologically by the 
fi nding of IgG, IgA and C

3 
deposited on the surface of 

the kera  nocytes.1,2,3,4 In the pa  ent's serum, indirect 
immunofl uorescence demonstrates the presence of 
circulating pemphigus autoantibodies that bind to 
epidermis. The  ter of circula  ng an  body correlates 
with disease course.5,6,7

Pat h o ge n e s i s  o f  p e m p h i g u s  i s  t h e  b i n d i n g  o f 
autoan  bodies to kera  nocyte cell surface molecules. 
These intercellular or pemphigus an  bodies bind to 
keratinocyte desmosomes and to desmosome-free 
areas of the kera  nocyte cell membrane. The binding 
of autoan  bodies results in blisters and a loss of cell-cell 
adhesion, a process termed acantholysis.8,9

Pemphigus has the tendency to recurrent and its clinical 
symptoms is varied. Prognosis is worse in patients 
with extensive lesion, especially when associated with 
infec  on.10 Therefore, the diagnosis and treatment has 
been one of the issues to focus on to the dermatologists. 
That is why we the choose pemphigus to study its 
features and treatment eff ect.

We analyzed and summarized the clinical manifesta  ons 
and the treatment of pemphigus to supply the reference 
of clinical diagnosis and treatment.

MATERIAL AND METHODS 
All pa  ents diagnosed as pemphigus from the out and 

in-pa  ent Department of Dermatology and Venereology 
of Lumbini Medical College were selected for the study. 
Their records were reviewed retrospec  vely. Pa  ents 
visi  ng from 1st of June 2009 to 31st of May 2013 were 
included in the study. 

Socio-Demographic data and subtype of disease 
were studies. All the data was entered into Microso   
Excel and sta  s  cal analyses were performed by the 
SPSS17.0 so  ware package and expressed with mean 
and standard devia  on.

RESULTS
There were a total of 70 pa  ents enrolled in the study. 
Out of those, 38(54%) were male and 32(46%) were 
female. The extent of pa  ent’s age was from 16 to 82 
years old (mean 41.3 ± 28.5).

Disease was classifi ed into following types:

1. Pemphigus vulgaris

2. Pemphigus vegetans

3. Pemphigus foliaceus

4. Pemphigus erythematosus

5. IgA pemphigus

6. Herpe  form  pemphigus

7. Paraneoplas  c pemphigus

There were 42  cases of Pemphigus vulgaris accoun  ng 
for 60% of all those cases. Sixteen cases (22.8%) were 
diagnosed as pemphigus foliaceus. Eight cases (11.4%) 
were of Pemphigus erythematosus variety. There were 
4 cases (5.8%) diagnoses as Pemphigus vegetans. There 



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Journal of Lumbini Medical College

were no cases of IgA, Paraneoplas  c and Herpe  form 
pemphigus.

Fig 1: Sex distribu  on

THERAPEUTIC EFFECT:
Pemphigus vulgaris: Out of 42 cases, 32 were clinically 
cured and 8 cases were improved. 

Pemphigus foliaceus: All the 16 cases were cured.

Pemphigus erythematosus: All 8 cases were cured.

Pemphigus vegetans: All 4 cases were cured as shown 
in table 2.

Table-1: Types of Pemphigus

Disease Number Percentage
Pemphigus vulgaris 42 60
Pemphigus vegetans 4 5.8
Pemphigus foliaceus 16 22.8
Pemphigus erythematosus 8 11.4
IgA pemphigus 0 0
Herpe  form  pemphigus 0 0
Paraneoplas  c pemphigus 0 0

Table 2: Results a  er treatment

Disease Total Cured Improved
Pemphigus vulgaris 42 34 8
Pemphigus vegetans 4 4 NA
Pemphigus foliaceus 16 16 NA
Pemphigus erythematosus 8 8 NA
IgA pemphigus NA NA NA
Herpe  form  pemphigus NA NA NA
Paraneoplas  c pemphigus NA NA NA

NA= Not available or Non applicable

DISCUSSION
Pemphigus is a worldwide disease and varies its 
presenta  on, clinical profi le, and incidence in diff erent 
regions of the world. In general, the incidence ranges 
from 0.76 to 5 new cases per million per year.11

There was a male dominance in our study with M:F ra  o 
of 1.18. Overall, the sex ra  o for pemphigus appears 
to be equivalent or close to equivalent. However, a 
few studies have found large imbalances in the sex 

distribu  on, such as a study that found a 4:1 ra  o of 
females to males with pemphigus foliaceus in Tunisia 
and a study that found a 19:1 ra  o of males to females 
in an endemic loca  on in Columbia.12,13

Pemphigus vulgaris was the most common variety in our 
study. This result was comparable to those reported in 
the majority of the studies.12,13

Treatment was very effective in early and limited 
diseases with 100% cure rate in many cases. Some 
advanced cases were par  ally improved. Similar results 
were shown by studies.14,15,16

CONCLUSIONS
Pemphigus is a common dermatological disease. It aff ects 
males more than females. Pemphigus vulgaris is the 
most common clinical type of Pamphigus, accoun  ng for 
60% of the pa  ents, followed by pemphigus foliaceus, 
accoun  ng for 22.8%. IgA pemphigus, Paraneoplas  c 
andherpe  form pemphigus are rare.

The total cure rate is 88.6%. Treatment is very eff ec  ve 
for vegetans, foliaceus and erythematosus types. 
Whereas it is eff ec  ve for Pemphigus vulgaris with 
cure rate of 81%. The eff ect of treatment in pa  ents 
with mild to moderate condi  on of pemphigus vulgaris, 
pemphigus foliaceus, pemphigus erythematosus and 
pemphigus vegetans was best, while in serious condi  on 
of pemphigus vulgaris it was par  al. 

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