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Cutaneous Manifestations of Systemic Lupus Erythematosus in Pakistani Patients M A

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Cutaneous Manifestations of Systemic Lupus Erythematosus in Pakistani Patients M A eCommons@AKU Section of Internal Medicine Department of Medicine November 2003 Cutaneous manifestations of systemic lupus erythematosus in Pakistani patients M A. Rabbani Aga Khan University S M A Shah Aga Khan University A Ahmed Aga Khan University Follow this and additional works at: https://ecommons.aku.edu/ pakistan_fhs_mc_med_intern_med Recommended Citation Rabbani, M. A., Shah, S. M., Ahmed, A. (2003). Cutaneous manifestations of systemic lupus erythematosus in Pakistani patients. Journal of Pakistan Medical Association, 53(11), 539-541. Available at: https://ecommons.aku.edu/pakistan_fhs_mc_med_intern_med/59 anti-D relapsed. Two patients after splenectomy and one and the duration of responses with international studies are after a second dose of anti-D. summarized in table 2. Follow up of patients who were resistant to anti-D Conclusion Five out of 11 patients were non responders to an It is concluded that anti-D is a relatively safe, initial infusion of anti-D. Three underwent splenectomy. convenient and effective therapy for chronic ITP and can be One received a second infusion of IV anti-D and remained used as a splenectomy sparing agent when treatment is in complete remission till last follow up. One patient was clinically indicated. A 50 µg /kg dose of anti-D yields more lost to follow up. than 50% response rates with median duration of effect of Discussion 12 weeks. Patients with ITP may require therapy to increase the References platelet counts for a variety of reasons and a number of 1. George JN, Woolf SH. Idiopathic thrombocytopenic purpura: a practice therapies are available to achieve that effect. However most guideline developed by explicit methods for the American Society of Hematology. Blood 1996; 88:3-40. of these therapies are helpful on short term basis and many 2. Salama A, Mueller-Eckhardt C. Effect of intravenous immunoglobulin in have unacceptable side effects and high costs. Anti-D immune thrombocytopenia competitive inhibition of reticuloendothelial appears to be promising since cost is low and side effects system functions by sequetration of autologous red blood cells? Lancet 1983; are minor and rare. It can be given on outpatient basis 2:193-5. 3. Waintraub S, Brody J. Use of anti D in immune thrombocytopenic purpura as saving admission costs and considerable morbidity a means to prevent splenectomy: case reports from two university hospital associated with other second line therapies. medical centers. Semin Hematol 2000;37 (suppl.1):S45-9. 4. Newman G, Novoa M, Fodero E. A dose of 75 microg/kg/d of i.v. anti-D In our study the responses were rapid, had with increases the platelet count more rapidly and for a longer period of time than minimal toxicity a reasonable duration of effect. 50microg/kg/d in adults with immune thrombocytopenic purpura. Br-J- Haematol 2001;112:1076-8. However in comparison to splenectomy the response 5. Andrew M, Blanchette VS, Adams M, et al. A multicentre study of the rates were significantly lower and durable responses were treatment of childhood chronic idiopathic thrombocytopenic purpura with seen in only 50% of the patients as compared to more than anti-D. J Pediatr 1991;120:522-7. 6. Michael D, Renee M, Lucille F, et al. Treatment of childhood acute immune 70% in the splenectomy arm. thrombocytopenic purpura with anti-D immune globulin or pooled immune Most of the available literature recommends anti-D globulin. J Pediatr 1999;134:21-6. 7. Scaradavou A, Woo B, Woloski BM, et al. Intravenous anti-D treatment of as splenectomy sparing therapy and our recommendation immune thrombocytopenic purpura: experience in 272 patients. Blood would remain such too. The comparison of response rates 1997;89:2689-700. Cutaneous Manifestations of Systemic Lupus Erythematosus in Pakistani Patients M. A. Rabbani, S. M. A. Shah, A. Ahmed Department of Medicine, The Aga Khan University Hospital, Karachi. 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). 539 J Pak Med Assoc Introduction lesions, 60% had cutaneous and systemic lesions and 30% had only systemic lesions. SLE is perhaps the best example of a multi system disorder in which cutaneous components of the disease can Table. Clinico-laboratory data of 196 patients with systemic lupus yield valuable diagnostic and prognostic information. erythematosus. Variations however exist in the incidence, clinical heterogeneity and severity of disease between different Clinico-laboratory features Positive Patients out of Total ethnic and racial groups. Environmental, cultural or genetic No. % backgrounds may explain these variations.1,2 The skin and mucous membranes are symptomatically involved at some ANA 191/198 96 point in over 80% of patients with SLE.3 There is a Anti dsDNA 146/198 74 tremendous variability and diversity in the type of Low C3 175/198 88 involvement ranging from classical butterfly rash and Anemia 134/189 70 atrophic hyperkeratotic lesions of discoid lupus to bullae, Leucopenia 38/189 20 alopecia and vasculitis or dermal vessels.4 Lymphopenia 101/189 53 For purpose of identifying patients in clinical studies Thrombocytopenia 48/189 25 American Rheumatology Association (ARA) revised Raised Serum Creatinine 52/189 27 criteria1 for classification of lupus is used. A person is said Proteinuria 48/198 24 to have SLE if any 4 or more of the 11 criteria are present, serially or simultaneously during any interval of CNS involvement 60/198 30 observation. Cutaneous lesions are important as a diagnostic Pulmonary involvement 33/198 17 aid as reflected by the fact that they account for four of the Cardiac involvement 24/198 12 11 revised ARA criteria of SLE. Skin and mucous membranes 140/198 70 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 Among LE-specific lesions noted were malar rash cutaneous lesions in SLE in Pakistani patients. (31%), discoid rash (15%), photosensitivity (33%) and mucopapular rash (20%). Bullae were not seen. Non- Patients and Methods specific lesions of SLE included vascular telangiectasia Record files of all patients between 1986 and 2001 (17%), micro infarcts (14%), palmar erythema (20%), who fulfilled the American Rheumatology Association chronic ulcers (3%), peripheral gangrene (2%), chilblains revised criteria for the classification of SLE1 were reviewed (1%), thrombophelibitis (2%), Raynaud`s phenomenon retrospectively. (2.5%), livedo reticularis (3%) and erythema multiform (1%). None of the patients had atrophae blanche, Using SPSS soft version (Release 8.0, standard rheumatoid nodules, erythromelalgia, sclerodactaly or version, copyright c SPSS; 1989-97), the patients were pyoderma gangreosum. Hyperpigmentation occurred in analyzed according to their age, sex, and clinical features 20% of patients. Hair Changes included noncicatricial with special attention to cutaneous manifestations. diffuse alopecia, cicatricial alopecia and lupus hair. Laboratory investigations included complete blood counts, serum creatinine, ESR, Serum total proteins, 24 hours Seven percent patients presented with nail changes, urinary proteins and creatinine clearance, anti nuclear and included ragged cuticles (3%), leukonychia (3%), factor, anti-DNA, Rheumatoid factor, serum compliment splinter hemorrhages (2%), paronychia (10%), nail fold levels, anti-ENA, skin biopsy, chest X-ray, ultrasound telangiectasia (5%) and onycholysis (7%). Bluish kidneys and echocardiogram. discoloration of nails was not observed in our series of patients. Results Oral mucosal lesions occurred in 21% of the Of the total 196 patients who fulfilled the American patients. Superficial erosions, discoid lesions and erythema Rheumatology Association revised criteria for SLE, 88% were noted on the lips, palate, buccal mucosa and gums. The were females and 12 % were male patients with male to rest of the mucosal surfaces of the body were not affected. female ratio of 1:8. Mean age at presentation was 31 years Other findings were localized and generalized pruritis (7%), (±12.3). Precipitating factors included sunlight (50%), Urticaria (10%), Acquired ichthyosis (1%) and acanthosis pregnancy (10%), drugs (15%) and infections (7.5%). At the nigricans (1%). Calcinosis, facial edema and panniculitis time of presentation only 10% patients had only cutaneous (5) were not recorded. Vol. 53, No. 11, November 2003 540 Infections noted were, herpes labialis (3%), herpes Khan10, 57% by Wysenbeek12 and 58% by Alarcon- zoster (2%), scabies (2%), furunculosis
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