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Discoid Lupus Erythematosus: A Profile

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ORIGINAL ARTICLE

Discoid Lupus Erythematosus: A Profile

Doulat Rai Bajaj1, Bikha Ram Devrajani2 and Bhajan Lal Matlani1

ABSTRACT
Objective: To determine the demographic data, clinical pattern and therapeutic outcome in patients with discoid lupus
erythematosus (DLE).
Study Design: Case series.
Place and Duration of Study: The Department of Dermatology, Liaquat University of Medical and Health Sciences,
Jamshoro, from January 2004 to December 2008.
Methodology: Patients of either gender aged above 18 years diagnosed with DLE were enrolled for the study. Those with
evidence of systemic lupus erythematosus were excluded. Apart from the onset, duration, symptoms, lesion’s location,
size and dimensions were noted. Biopsy was taken when the diagnosis was in doubt. Apart from routine investigations
serum anti-nuclear factor was determined in every patient. The data were analyzed using SPSS software version 11.0 for
frequencies and percentages.
Results: There were 110 patients (38 males and 72 females), with ages between 18 and 62 years. Family history was
positive in 3 patients. The plaque form was the most common clinical type seen in 68 (61.8%) patients, followed by tumid
(n=20, 18.2%), panniculitis (n=10, 9.1%) and ulcerative (n=8,7.3%) types. Face was the most common site affected (n=60,
54.5%). Antinuclear antibody was present in 19 (17.3%) patients. Fatigue and joint pains were the commonest symptoms
(n=52, 47%). Pigmentation and scarring were the usual outcome.
Conclusion: DLE is a chronic disease with multiple presentations, which usually ends with pigmentation and scarring.

Key words: Discoid lupus erythematosus. Systemic lupus erythematosus. Autoimmune disease. Antinuclear antigens. Clinical pattern.

INTRODUCTION However, considering the frequency of patients being

Discoid lupus erythematosus (DLE) is an autoimmune
disease characterized by well-defined inflammatory,
scaly plaques on skin.1 The lesions most frequently
involve sun-exposed areas like face, neck, ears and
upper trunk.2 The disease may occur at any age; with
higher incidence between 20 to 40 years of age.3 DLE
is essentially a cutaneous disease with negligible
propensity for systemic organ involvement. The most
common clinical presentation is an erythematosus,
centrally atrophic plaque with surface telangiectasia and
pigmented borders.4 Its less frequent presentations
include tumid, warty, chillblain, telangiectatic rosaceous,
ulcerative, linear, bullous and acneiform.5-7 The lesions
are localized to head and neck in majority of cases.
Generalized involvement have been reported in 50% of
cases.8
In a hospital-based data from England, there were
0.003% of out-patient consultations for DLE during
2002-2003.9 Its prevalence in Pakistan is unknown.
1 Department of Dermatology, Liaquat University of Medical and
Health Sciences (LUMHS), Jamshoro.
2 Medical Unit-III, Liaquat University of Medical and Health
Sciences (LUMHS), Jamshoro.
Correspondence: Dr. Doulat Rai Bajaj, Bangalow No. A-12,
Bagh-Muhammad Housing Scheme, Opposite Grid Station,
Qasimabad, Hyderabad.
E-mail: doulat01@yahoo.com
Received June 22, 2009; accepted March 24, 2010.
seen at primary and tertiary care hospitals, it can not be
considered an uncommon disease in our population.
The demographic and other data regarding pattern of
disease and the risk factors are unknown in our
population. An accurate knowledge of this data is
important so that the disease is recognized at its early
stages. This would enable physician to manage it and
take preventive measures earlier to minimize subsequent
complications. Keeping this objective in mind, this study
was conducted.
The aim of this study was to assess the demographic
data, clinical pattern, evolution, and outcome of treatment
in patients with DLE presenting at a tertiary hospital.
METHODOLOGY
This observational study was conducted at the
Department of Dermatology, Liaquat University of
Medical and Health Sciences, Jamshoro, over a period
of 5 years from January 2004 to December 2008.
All patients aged above 18 years, of either gender,
clinically diagnosed as cases of DLE, were enrolled for
study. An informed consent was sought from them after
due explanation of the purpose and procedure. The
study was approved by local ethical committee. Non-
willing patients and those with systemic manifestations
suggestive of systemic lupus erythematosus or any
chronic systemic disease were excluded from study.
History included; bio-data of patients (name, age,
gender, address and occupation), symptoms, duration,

Journal of the College of Physicians and Surgeons Pakistan 2010, Vol. 20 (6): 361-364 361
Doulat Rai Bajaj, Bikha Ram Devrajani and Bhajan Lal Matlani

and evolution of their disease. Particular emphasis was The plaque form was the most common clinical type
laid on occupation, leisure activities, outdoor hobbies seen in 68 (61.8%) patients, which included 24 males
and drug intake in recent past. A family history of and 44 females. This was followed by tumid (20, 18.2%),
disease was also sought. The patients were examined panniculitis (10, 9.1%), ulcerative (8, 7.3%), and
each time by two dermatologists. The diagnosis was rosaceous (4, 3.6%) types. The most common site
made on clinical features of DLE i.e. inflammatory affected was face (n=60, 54.5%). The disease was
plaques, central atrophy, follicular plugging, pigmentary generalized in nature in only 20 (18.2%) patients. In
disturbances in form of hyper or hypopigmentation, pre-
dilection for sun-exposed areas, symmetrical distribution,
evidence of solar damage e.g. lentigines, freckles,
keratotic papules etc. Biopsy was done where the
diagnosis was doubtful due to the lack of typical
features. All patients were also examined by a qualified
physician for evaluation of systemic disease.
Apart from routine investigations like complete blood
counts, urine analysis, serum electrolytes, liver profile,
all the patients had their serum analyzed for anti-nuclear
factor (ANA). Patients having generalized lesions with
vague general symptoms were also advised serum anti-
ds-DNA to exclude occult systemic lupus erythematosus
(SLE). Age group

The lesions were examined for location, nature,

Figure 1: Age and gender distribution of patients.
dimensions, depth and secondary changes. The lesions
on cheeks, forehead, nose, lips (outside the vermillion
Table I: Demographic and clinical data of patients.
border) and ears were grouped as facial. In case of
Male Female Total
lesions on more than one site (but not more than 3), the
n= 38 (34.5) n=72 (65.4) n= 110 (%)
site bearing the maximum number or having larger
Inhabitance
lesion was used to classify location. DLE was classified Rural 12 62 76 (69)
as localized when lesions were situated over head and Urban 24 10 34 (31)
neck, and generalized when these were more Education
widespread below neck. Localized disease was again Non-educated 6 40 46 (41.8)
classified into mild (number of lesions less than 5), School 18 30 48 (43.6)
moderate (5-9 lesions) and severe (more than 10 College 10 02 12 (10.9)
lesions). Mild disease was treated with sunscreens and University 4 00 04 (3.6)
topical steroids. In moderate and severe disease oral Occupation
antimalarials and steroids were also added. None 06 40 46 (41.8)
Labourer 10 24 34 (30.9)
All the data were entered into a closed end proforma Farmer 12 08 20 (18.2)
and analyzed using SPSS version 11.0. Qualitative data Small business 06 00 06 (5.5)
i.e. gender, clinical types of DLE, sites were presented Official 04 00 04 (3.6)
as number (n) and percentages. Type of Lesion
Plaque 24 44 68 (61.8)

RESULTS Tumid 6 14 20 (18.2)

Panniculitis 2 8 10 (9.1)
Of the 110 patients seen, 72 (65.5%) were females and Ulcerative 4 4 8 (7.3)
38 (34.5%) males; with a male to female ratio of 1:1.9. Rosaceous 2 2 4 (3.6)
Figure 1 shows the age and gender distribution of Site of Lesion
patients. The mean age at onset was 31.40 ± 9.57 years Face 22 38 60 (54.5)
ranging from 18 to 61 years. Seventy eight percent of Scalp 10 16 26 (23.6)
patients were between 21 to 40 years age, with Neck 2 8 10 (9.1)
maximum number (24, 44%) between 21-30 years. Upper chest 4 4 8 (7.3)
Hands and feet 0 6 6 (5.5)
Twenty seven patients were regular smokers, 22 males
Extent
and 05 females were smoking more than 10 cigarettes.
Localized 34 56 90 (81.8)
Eighteen patients were rural dwellers.
Generalized 4 16 20 (18.2)
The demographic data and characteristics of disease Antinuclear Antibodies (ANA) 04 15 19 (17.3)
are shown in Table I. n=number (%).

362 Journal of the College of Physicians and Surgeons Pakistan 2010, Vol. 20 (6): 361-364
Discoid lupus erythematosus
remaining it was localized to sites shown in Table I. The
disease was graded as mild in 53, moderate in 33 and
severe in 24 patients. Fifty four patients (49%) had sun
exposure for 5-6 hours per day due to their work/job.
Among those, 15 were males and 12 females. Forty
female and 6 male patients were non-educated (Table I).
Antinuclear antibody was present in 19 (17.3%) patients.
The titre was markedly raised (1/160) in only 2 patients.
Both were females. In the remainder, the titre was low;
from 1/10 dilution to 1/50 dilution. Homogenous pattern
was more common (n=9,47.36%), followed by speckled
(n=5,26.3%) pattern. In remainder the pattern remained
unknown.
Fatigue and joint pains were the commonest symptoms
(n=52, 47%) seen mostly in females (n=47).
Episodic ordinary urticaria (n=14) and alopecia areata
(n=7) were the co-morbid conditions found in this study.
Residual pigmentation and scarring was the most
common outcome in virtually all patients except those
with the panniculitis form.
The patients with mild disease (having small lesions
measuring < 3 cms and less than 5 in number) were
treated with one of the moderately potent topical steroid
creams and ointments as per suitability. Those with
more lesions (5-9) were treated with oral hydroxy-
chloroquin (200 mg twice a day) with topical steroids. All
the patients with mild disease (n=53) responded well to
topical steroids. Low dose prednisolone (10 mg/d) was
prescribed to only 05 patients, in addition to oral
chloroquin and topical steroids. A sunscreen with SPF
60 with oral antioxidants containing β-carotene was
always part of regimen. Patients were followed for 4
years and none developed frank SLE.
DISCUSSION
Discoid lupus erythematosus is essentially a cutaneous
disease with different genetic and phenotypic charac-
teristics from systemic lupus erythematosus (SLE).
Discoid lesions have been documented as a feature of
SLE in many studies conducted at home. There has
been no attempt to describe DLE as a separate disease
entity in our population. Keeping this in mind we sought
to explore the clinical and demographic features of DLE.
The female preponderance seen in this study is
consistent with other studies.10 But it contrasts with the
study by Ng et al. in which the males outnumbered
females.2 However, the number of male and female
patients were equal at the 6th decade in this study. This
feature also correlates with other studies.11 Hormonal
factors such as estrogen are considered additional risk
factors in females.12 The mean age of presentation of
31.40 ± 9.57 years in the patients of this study
coinciding with the age range given in another study, but
it is slightly younger than that (34.1±15.1 years) reported
by Tebbe and Ng et al.12,13
Journal of the College of Physicians and Surgeons Pakistan 2010, Vol. 20 (6): 361-364
These slight differences may be due to genetic and
environmental factors.14 Our population is exposed to
ultraviolet radiation (UVR) due to their job/work. A high
number of patients in this study were either jobless or
doing those jobs (e.g. farming, labour on daily wages,
small business) that exposed them to higher quantity of
UVR. The jobless females were housewives doing
domestic work in UVR-unprotected environment at their
homes.
Two thirds of the patients belonged to rural areas living
in open houses with poor protection from UVR. These
factors exposed them constantly to UVR for long hours.
UV light induces apoptosis of keratinocytes with
production and release of pro-inflammatory cytokines.
These cytokines induce inflammatory changes in
susceptible individuals.15,16 Moreover, the high incidence
of disease between ages 20-40 (peak age of working
people) also highlights the stronger role of UVR in
causing disease in the local population. HLA studies
need to be done to find the genotype characteristics of
our population to see the extent, to which the studied
population genetically predisposed to DLE.17
There was higher incidence of disease in patients who
were either non-educated or educated upto primary
school level as compared to those who had high
school/college/university education. Illiteracy leads to
unemployment and poverty. These coupled with un-
awareness of hazards of sun exposure which, as a
result increase the incidence in low socioeconomic
group. However, larger controlled studies are needed to
further elucidate this phenomenon.
Plaque form was the most common type of presentation
in this study. This is in accordance with other studies.18
The marked pigmentary changes in lesions was
significant in patients. This feature is peculiar to Asian
population.19 The skin type of Pakistani population
(type-III to type-V), is associated with general tendency
to postinflammatory melanosis and these are are the
contributing factors for residual pigmentation of
lesions.20
Similarly, face and scalp were the most common sites
affected. Khelifa et al. documented lesions on face in 25
of his 26 patients. The disease was localized in more
than 3/4th of patients. These features may be attributed
to sun exposure.
ANA were present in a small number of patients; mostly
females. Only 2 female patients had significant titres.
This was expected as most of the previous studies have
detected this antibody in a few patients with DLE and in
low titres. ANA was significant in those patients who had
generalized disease. The disease was present for more
than 05 years in these patients. They also complained of
fatigue and arthralgias, but there was no clinical
or serological evidence of SLE in those patients. The
363
Doulat Rai Bajaj, Bikha Ram Devrajani and Bhajan Lal Matlani
conversion to systemic disease is observed in those
patients who had severe disease at the onset with
higher titres of ANA.21 The most common pattern of
ANA was homogenous. This contrasts with the Ng et al.
in which the dominant pattern was speckled.2,13 Patients
were followed for 4 years and none of the patients in this
study transformed to frank SLE.
Pigmentation and scarring of some degree, were the
chief complications virtually present in all the patients in
this study. However, the frequency of these complications
is twice the figures given by de Berker et al.22 This again
may be attributed sun exposure in these patients.
The treatment strategy employed was very similar to
that used by Panjwani et al.3 There was satisfactory
improvement in mild to moderately severe disease in
this study, as was also demonstrated in the latter study.
However, immunosuppressive drugs like azathioprine
and methotrexate were used for severe disease in a
study conducted at Singapore.2 There was no patient
with severe disease in this study, therefore, no need of
using these immunosuppressives.
CONCLUSION
DLE is a chronic disease with multiple presentations.
Sun protection measures must be advised to patients to
lessen the risk of disease. In the local population, there
is a high chance of residual pigmentation and scarring,
therefore, its early recognition and management is
essential to prevent disfigurement.
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Journal of the College of Physicians and Surgeons Pakistan 2010, Vol. 20 (6): 361-364