November 2003, Volume 53, Issue 11

Original Article

Cutaneous Manifestations of Systemic Lupus Erythematosus in Pakistani Patients

M. A. Rabbani  ( Department of Medicine, The Aga Khan University Hospital, Karachi. )
S. M. A. Shah  ( Department of Medicine, The Aga Khan University Hospital, Karachi. )
A. Ahmed  ( Department of Medicine, The Aga Khan University Hospital, Karachi. )

Introduction

SLE is perhaps the best example of a multi system disorder in which cutaneous components of the disease can yield valuable diagnostic and prognostic information. Variations however exist in the incidence, clinical heterogeneity and severity of disease between different ethnic and racial groups. Environmental, cultural or genetic backgrounds may explain these variations.1,2 The skin and mucous membranes are symptomatically involved at some point in over 80% of patients with SLE.3 There is a tremendous variability and diversity in the type of involvement ranging from classical butterfly rash and atrophic hyperkeratotic lesions of discoid lupus to bullae, alopecia and vasculitis or dermal vessels.4
For purpose of identifying patients in clinical studies American Rheumatology Association (ARA) revised criteria1 for classification of lupus is used. A person is said to have SLE if any 4 or more of the 11 criteria are present, serially or simultaneously during any interval of observation. Cutaneous lesions are important as a diagnostic aid as reflected by the fact that they account for four of the 11 revised ARA criteria of SLE.
Data on the cutaneous features of SLE in Pakistan seems somewhat scarce. The main purpose of this study was to analyze the clinical importance and prevalence of cutaneous lesions in SLE in Pakistani patients.

Patients and Methods

Record files of all patients between 1986 and 2001 who fulfilled the American Rheumatology Association revised criteria for the classification of SLE1 were reviewed retrospectively.
Using SPSS soft version (Release 8.0, standard version, copyright c SPSS; 1989-97), the patients were analyzed according to their age, sex, and clinical features with special attention to cutaneous manifestations. Laboratory investigations included complete blood counts, serum creatinine, ESR, Serum total proteins, 24 hours urinary proteins and creatinine clearance, anti nuclear factor, anti-DNA, Rheumatoid factor, serum compliment levels, anti-ENA, skin biopsy, chest X-ray, ultrasound kidneys and echocardiogram.

Results

Of the total 196 patients who fulfilled the American Rheumatology Association revised criteria for SLE, 88% were females and 12 % were male patients with male to female ratio of 1:8. Mean age at presentation was 31 years (±12.3). Precipitating factors included sunlight (50%), pregnancy (10%), drugs (15%) and infections (7.5%). At the time of presentation only 10% patients had only cutaneous lesions, 60% had cutaneous and systemic lesions and 30% had only systemic lesions.
Among LE-specific lesions noted were malar rash (31%), discoid rash (15%), photosensitivity (33%) and mucopapular rash (20%). Bullae were not seen. Non-specific lesions of SLE included vascular telangiectasia (17%), micro infarcts (14%), palmar erythema (20%), chronic ulcers (3%), peripheral gangrene (2%), chilblains (1%), thrombophelibitis (2%), Raynaud`s phenomenon (2.5%), livedo reticularis (3%) and erythema multiform (1%). None of the patients had atrophae blanche, rheumatoid nodules, erythromelalgia, sclerodactaly or pyoderma gangreosum. Hyperpigmentation occurred in 20% of patients. Hair Changes included noncicatricial diffuse alopecia, cicatricial alopecia and lupus hair.
Seven percent patients presented with nail changes, and included ragged cuticles (3%), leukonychia (3%), splinter hemorrhages (2%), paronychia (10%), nail fold telangiectasia (5%) and onycholysis (7%). Bluish discoloration of nails was not observed in our series of patients.
Oral mucosal lesions occurred in 21% of the patients. Superficial erosions, discoid lesions and erythema were noted on the lips, palate, buccal mucosa and gums. The rest of the mucosal surfaces of the body were not affected. Other findings were localized and generalized pruritis (7%), Urticaria (10%), Acquired ichthyosis (1%) and acanthosis nigricans (1%). Calcinosis, facial edema and panniculitis (5) were not recorded.
Infections noted were, herpes labialis (3%), herpes zoster (2%), scabies (2%), furunculosis and folliculitis (4%), tinea corporis (7%), cellulitis and abscess (5%) and oral candidiasis (12%).
Systemic involvement was present in 90% patients and included arthritis (38%), nephritis (36%), pericarditis (12%), lung involvement (17%) and CNS involvement (30%). 83% Patients were found to have hematological disturbances with anemia (71%), leukopenia (20%), lymphopenia (53%) and thrombocytopenia (26%). ESR was raised in nearly 100% patients. Other positive laboratory findings included positive ANA (93%), anti dsDNA (83%), low C3 (85%), low C4 (41%), protienuria (24%), and RBC and casts in the urine (32%).

Conclusion

Cutaneous lesions in SLE are important as a diagnostic aid as reflected by the fact that they account for four of the 11 revised American Rheumatism Association criteria of SLE. The pattern and incidence of skin changes may vary from place to place.

References

1. Tan EM, Cohen AS, Fries JF, et al. The 1982 revised criteria for classification of SLE. Arthritis Rheum 1982;25:1271-7.

2. Font J, Bosch X, Ingelmo M, et al. Acquired ichthyosis in a patient with SLE.Arch Dermatol 1990;126-9.

3. McCauliffe DP. Cutaneous lupus erythematosus:. Semin Cutan Med Surg. 2001;20: 14-26.

4. Laman SD, Provost TT. Cutaneous manifestations of SLE. Rheum Dis Clin North Am 1994;20:195.

5. Hochberg MC, Boyd RE, Ahearn JM, et al. SLE a review of clinico-laboratory features and immunogenetic markers in 150 patients with emphasis on demographic subsets. Medicine 1985;64:285-95.

6. Watson R. Cutaneous lesions in SLE. Med Clin North Am 1989;73:1091-1109.

7. Kapadia N, Haroon TA. Cutaneous manifestations of systemic lupus erythematosus. Int J Dermatol 1996;408-9.

8. George R, Mathai R, Kurain S. Cutaneous lupus erythematosus in India. Immunoflouresence profile. Int J Dermatol 1992;31:265-8.

9. Weinstein C, Miller MH, Axtens R, et al. Livido reticularis associated with increased titres of anti cardiolipin antibodies in SLE. Arch Dermatol 1987;123:596-600.

10. Akhter J, Khan MA. SLE in Pakistan. Specialist 1992; 9:25-7.

11. Tuffanelli DL, Dubois EL. Cutaneous manifestations of SLE. Arch Dermatol 1964; 90:377-86.

12. Wysenbeek AJ, Leibovici L, Amit M, et al. Alopesia in SLE. J Rheumatol 1991; 18:1185-6.

13. Alargon SD, Cetina JA. Lupus hair. Am J Med Sci 1974;267:241-2.

14. Rothfield NF. SLE clinical aspects and treatment. In: McCarty DJ, ed. Arthritis and allied conditions. Philadelphia: W.B. Saunders, 1990, pp.183-6.

15. Noz KC. ANA-negative SLE. Br J Dermatol 1993;129:345-6.

16. McCarty GA. Autoantibodies and their relation to rheumatic disease. Advances in rheumatology. Med Clin North Am 1986;70:237-56.

Abstract

Objective: Systemic Lupus Erythematosus (SLE) is an autoimmune process in which cutaneous lesions occur in majority of patients. This study from Karachi, Pakistan was conducted to determine the pattern and prevalence of such lesions in SLE in Pakistani patients.

Methods: One hundred ninety eight patients with SLE fulfilling the clinical and laboratory criteria of the American Rheumatology Association were examined between 1986 and 2001` for the presence of cutaneous manifestations.
Results: Skin changes noted were: noncicatricial diffuse alopecia (22%), malar rash (31%), mucosal lesions (20%), discoid eruptions (15%), photosensitivity (33%), vascular lesions (20%), pruritis (17%), and pigmentary changes (22%). Peripheral gangrene,chronic ulcers, Raynauds phenomenon, urticaria, chilblains, thrombophlebitis, palmar erythema, and erythema multiform were rare. Anti ANA and anti dsDNA were positive in 93% and 83% patients respectively.

Conclusion: A different clinical pattern was noted in our patients than reported previously (JPMA 53:539;2003).

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