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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.
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
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 These slight differences may be due to genetic and
disease was graded as mild in 53, moderate in 33 and environmental factors.14 Our population is exposed to
severe in 24 patients. Fifty four patients (49%) had sun ultraviolet radiation (UVR) due to their job/work. A high
exposure for 5-6 hours per day due to their work/job. number of patients in this study were either jobless or
Among those, 15 were males and 12 females. Forty doing those jobs (e.g. farming, labour on daily wages,
female and 6 male patients were non-educated (Table I). small business) that exposed them to higher quantity of
Antinuclear antibody was present in 19 (17.3%) patients. UVR. The jobless females were housewives doing
The titre was markedly raised (1/160) in only 2 patients. domestic work in UVR-unprotected environment at their
Both were females. In the remainder, the titre was low; homes.
from 1/10 dilution to 1/50 dilution. Homogenous pattern Two thirds of the patients belonged to rural areas living
was more common (n=9,47.36%), followed by speckled in open houses with poor protection from UVR. These
(n=5,26.3%) pattern. In remainder the pattern remained factors exposed them constantly to UVR for long hours.
unknown. UV light induces apoptosis of keratinocytes with
Fatigue and joint pains were the commonest symptoms production and release of pro-inflammatory cytokines.
(n=52, 47%) seen mostly in females (n=47). These cytokines induce inflammatory changes in
susceptible individuals.15,16 Moreover, the high incidence
Episodic ordinary urticaria (n=14) and alopecia areata
(n=7) were the co-morbid conditions found in this study. of disease between ages 20-40 (peak age of working
Residual pigmentation and scarring was the most people) also highlights the stronger role of UVR in
common outcome in virtually all patients except those causing disease in the local population. HLA studies
with the panniculitis form. need to be done to find the genotype characteristics of
our population to see the extent, to which the studied
The patients with mild disease (having small lesions population genetically predisposed to DLE.17
measuring < 3 cms and less than 5 in number) were
treated with one of the moderately potent topical steroid There was higher incidence of disease in patients who
creams and ointments as per suitability. Those with were either non-educated or educated upto primary
more lesions (5-9) were treated with oral hydroxy- school level as compared to those who had high
chloroquin (200 mg twice a day) with topical steroids. All school/college/university education. Illiteracy leads to
the patients with mild disease (n=53) responded well to unemployment and poverty. These coupled with un-
topical steroids. Low dose prednisolone (10 mg/d) was awareness of hazards of sun exposure which, as a
prescribed to only 05 patients, in addition to oral result increase the incidence in low socioeconomic
chloroquin and topical steroids. A sunscreen with SPF group. However, larger controlled studies are needed to
60 with oral antioxidants containing β-carotene was further elucidate this phenomenon.
always part of regimen. Patients were followed for 4 Plaque form was the most common type of presentation
years and none developed frank SLE. in this study. This is in accordance with other studies.18
The marked pigmentary changes in lesions was
DISCUSSION significant in patients. This feature is peculiar to Asian
Discoid lupus erythematosus is essentially a cutaneous population.19 The skin type of Pakistani population
disease with different genetic and phenotypic charac- (type-III to type-V), is associated with general tendency
teristics from systemic lupus erythematosus (SLE). to postinflammatory melanosis and these are are the
Discoid lesions have been documented as a feature of contributing factors for residual pigmentation of
SLE in many studies conducted at home. There has lesions.20
been no attempt to describe DLE as a separate disease
Similarly, face and scalp were the most common sites
affected. Khelifa et al. documented lesions on face in 25
entity in our population. Keeping this in mind we sought
to explore the clinical and demographic features of DLE.
of his 26 patients. The disease was localized in more
The female preponderance seen in this study is than 3/4th of patients. These features may be attributed
consistent with other studies.10 But it contrasts with the
study by Ng et al. in which the males outnumbered
to sun exposure.
females.2 However, the number of male and female ANA were present in a small number of patients; mostly
patients were equal at the 6th decade in this study. This females. Only 2 female patients had significant titres.
feature also correlates with other studies.11 Hormonal This was expected as most of the previous studies have
factors such as estrogen are considered additional risk detected this antibody in a few patients with DLE and in
factors in females.12 The mean age of presentation of low titres. ANA was significant in those patients who had
31.40 ± 9.57 years in the patients of this study generalized disease. The disease was present for more
coinciding with the age range given in another study, but than 05 years in these patients. They also complained of
it is slightly younger than that (34.1±15.1 years) reported fatigue and arthralgias, but there was no clinical
by Tebbe and Ng et al.12,13 or serological evidence of SLE in those patients. The
Journal of the College of Physicians and Surgeons Pakistan 2010, Vol. 20 (6): 361-364 363
Doulat Rai Bajaj, Bikha Ram Devrajani and Bhajan Lal Matlani
conversion to systemic disease is observed in those 6. Dekle CL, Mannes KD, Davis LS, Sangueza OP. Lupus tumidus.
patients who had severe disease at the onset with J Am Acad Dermatol 1999; 41:250-3.
higher titres of ANA.21 The most common pattern of
ANA was homogenous. This contrasts with the Ng et al.
7. Kuhn A, Bijl M. Pathogenesis of cutaneous lupus erythematosus.
Lupus 2008; 17:389-93.
in which the dominant pattern was speckled.2,13 Patients 8. Deruelle-Khazaal R, Ségard M, Cottencin-Charrière AC,
were followed for 4 years and none of the patients in this Carotte-Lefebvre I, Thomas P. [Chronic lupus erythematosus
study transformed to frank SLE. presenting as acneiform lesions]. Ann Dermatol Venereol 2002;
129:883-5. French.
Pigmentation and scarring of some degree, were the
9. Cure Research. Epidemiological data regarding various
chief complications virtually present in all the patients in diseases in England [Internet]. [updated 2009 May 25]. Available
this study. However, the frequency of these complications
is twice the figures given by de Berker et al.22 This again
from: www.cureresearch.com
10. Francès C, Barète S, Piette JC. [Classification of dermatologic
manifestations in lupus erythematosus]. Rev Med Interne 2008;
may be attributed sun exposure in these patients.
The treatment strategy employed was very similar to 29:701-9. French.
that used by Panjwani et al.3 There was satisfactory 11. Lin JH, Dutz JP, Sontheimer RD, Werth VP. Pathophysiology of
improvement in mild to moderately severe disease in cutaneous lupus erythematosus. Clin Rev Allergy Immunol 2007;
this study, as was also demonstrated in the latter study. 33:85-106.
However, immunosuppressive drugs like azathioprine 12. Tebbe B, Orfanos CE. Epidemiology and socioeconomic impact
and methotrexate were used for severe disease in a of skin disease in lupus erythematosus. Lupus 1997; 6:96-104.
study conducted at Singapore.2 There was no patient 13. Ng PP, Tan SH, Koh ET, Tan T. Epidemiology of cutaneous lupus
with severe disease in this study, therefore, no need of erythematosus in a tertiary referral centre in Singapore. Australas
using these immunosuppressives. J Dermatol 2000; 41:229-33.
14. Kuhn A, Sontheimer RD. Cutaneous lupus erythematosus:
CONCLUSION molecular and cellular basis of clinical findings. Curr Dir
DLE is a chronic disease with multiple presentations. Autoimmun 2008; 10:119-40.
Sun protection measures must be advised to patients to 15. Furukawa F, Muto M. Ethnic differences in immunogenetic
lessen the risk of disease. In the local population, there features and photosensitivity of cutaneous lupus erythematosus.
is a high chance of residual pigmentation and scarring, Arch Dermatol Res 2009; 301:111-5. Epub 2008 Sep 17.
therefore, its early recognition and management is 16. Kuhn A, Sticherling M, Bonsmann G. Clinical manifestations of
essential to prevent disfigurement. cutaneous lupus erythematosus. J Dtsch Dermatol Ges 2007;
5:1124-37.
REFERENCES 17. Donnelly AM, Halbert AR, Rohr JB. Discoid lupus erythematosus.
Australas J Dermatol 1995; 36:3-10.
1. Burns T, Breathnach S, Cox N, Griffiths C, editors. Rook's
textbook of dermatology. 7th ed. San Francisco: Wiley-Blackwell; 18. Hymes SR, Jordon RE. Chronic cutaneous lupus erythematosus.
2004. Med Clin North Am 1989; 73:1055-71.
19. George R, Mathai R, Kurian S. Cutaneous lupus erythematosus
in India: immunofluorescence profile. Int J Dermatol 1992; 31:
2. Ng SK, Ratnam KV, Tan T. Discoid lupus erythematosus in
Singapore. Singapore Med J 1985; 26:465-8.
265-9. Comments in: Int J Dermatol 1993; 32:76.
3. Panjwani S. Early diagnosis and treatment of discoid lupus
erythematosus. J Am Board Fam Med 2009; 22:206-13. 20. Kapadia N, Haroon TA. Cutaneous manifestations of systemic
lupus erythematosus. Int J Dermatol 1996; 35:408-9.
4. Seitz CS, Bröcker EB, Trautmann A. Linear variant of chronic
cutaneous lupus erythematosus: a clue for the pathogenesis of 21. Patel P, Werth V. Cutaneous lupus erythematosus: a review.
chronic cutaneous lupus erythematosus? Lupus 2008; 17:1136-9. Dermatol Clin 2002; 20:373-85.
5. Pramatarov KD. Chronic cutaneous lupus erythematosus: 22. de Berker D, Dissaneyeka M, Burge S. The sequelae of chronic
clinical spectrum. Clin Dermatol 2004; 22:113-20. cutaneous lupus erythematosus. Lupus 1992; 1:181-6.
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364 Journal of the College of Physicians and Surgeons Pakistan 2010, Vol. 20 (6): 361-364