THROMBOCYTOPENIA AS A PRESENTING FEATURE OF SYSTEMIC LUPUS ERYTHEMATOSUS (SLE)
Karuna Thapar, Gaurav Dhawan
Department of Pediatrics, Government Medical College , Amritsar
Address for Correspondence
Karuna Thapar, Hno. 9-A, Krishna Square, Near Shivala Mandir. Amritsar, Punjab 143001.
Email
kthapar2000@yahoo.com
Introduction
Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by body's production of antibodies to nuclear components of cells and multiple target organ damage. Clinical presentation includes symptoms like fever, malaise, rash, oral ulcers, non-erosive inflammatory polyarthritis and malar rash. Here we report a case because of its atypical presentations.
Case Report
9 year old male presented to us with complaints of dryness of skin with fissuring and oozing of blood since 2 months of age. The skin lesions were predominantly confined to exposed areas, used to occur more during summers and were associated with exposure to sun. There was history of ecchymosis since 7 months of age usually after trauma with prolonged bleeding. There was history of 3 episodes of generalized seizures in the past 1.5 years. At presentation, vitals were stable and skin lesions having dryness and scaling were seen over face, neck and hands (Picture 1). There were petechiae on face and ecchymotic spots on left upper arm and legs with oral ulcers.



Hemoglobin was 6 gm%, Total leucocyte count, Differential leucocyte count were within normal limits, peripheral blood film was normal. Platelet count was decreased (35,000/cu mm). Bone marrow aspiration was done which showed erythroid hyperplasia with megaloblastic changes and increased megakaryocytes. In view of photosensitivity, oral ulcers, neurological involvement, and thrombocytopenia, diagnosis of SLE was suspected by fulfilling 4 out of 11 criterion established by American College of Rheumatology. Diagnosis was then confirmed by anti dsDNA level of 84.62 (Normal range < 60). Child was put on oral prednisolone. Platelet count rose to one lakh after 6 days of treatment with gradual improvement of his symptoms. Patient was given low dose of prednisolone that was tapered over the next 6 months. Patient showed good recovery and is still under follow up.
Discussion
Systemic Lupus Erythematosus (SLE) is a chronic, usually life-long, potentially fatal autoimmune disease characterized by unpredictable exacerbations and remissions with protean clinical manifestations. In SLE there is a predilection for clinical involvement of the joints, skin, kidney, brain, serosa, lung, heart and gastrointestinal tract. The incidence of SLE is reported to be higher in Black, Asian and Indian populations (1). SLE occurs in adult and pediatric populations, with nearly 15 percent of cases present in children younger than age 16 years. As reported by Caeiro, fewer than 26 percent of patients with SLE have an onset before age 10 years (2). Adult-onset patients have more frequent cardiovascular manifestations, whereas childhood-onset patients have more frequent hematological manifestations (3). General clinical features in childhood SLE include broad variations between presence of rash, arthritis, constitutional symptoms, renal disease, cardiovascular, pulmonary, and neuropsychiatric involvement (5). The most common clinical manifestations and the most common presenting symptoms of SLE are polyarthritis and dermatitis (6). However, any symptom or sign of the disease may be its first manifestation, and a single one, such as arthritis, thrombocytopenia, or pericarditis, may persist or recur for months or years before the diagnosis can be confirmed by the appearance of other features. Chronic fatigue and a variety of disturbances of cognition or affect, including anxiety and depression, are frequently described by patients as early symptoms (7).

The cutaneous and mucous-membrane manifestations of SLE are varied. They bear no consistent relation to disease activity in other organs (8). The classic butterfly malar erythema occurs in only one third of patients. A patchy maculopapular rash on the upper trunk and areas exposed to the sun (such as the neck and extensor surfaces of the arms and legs) is probably more common (9). A distinctive rash, referred to as subacute cutaneous lupus erythematosus, consists of chronic erythematous, papulosquamous patches on the trunk and limbs, and frequently on the face and palms as well (10).

Recurrent non-infectious pharyngitis and oral ulcers are common (11). Mucocutaneous manifestations in children with SLE include rash (76 %), photosensitivity (28 %), alopecia (55 %), Raynaud phenomenon (31 %), vasculitis (54 %), oral ulcers (19 %), purpura (16 %) and discoid lesions (7 %) (2). Comparison of the autoantibody profiles of adult and childhood-onset cases reveals a higher frequency of elevated anti-DNA antibodies in children (12).

The neurological complications of SLE can affect any part of the nervous system (13). Neuropsychiatric manifestations are reported in 29-44 percent of pediatric patients with SLE (14). The most common neurological symptoms in children include headaches, coma, psychosis and depression.

Treatment is determined to a great extent by individual disease manifestations rather than the primary diagnosis. There is no evidence that prophylactic treatment with a low dose of a glucocorticoid is beneficial. A prominent cause of death later in the course of disease is coronary atherosclerosis, possibly related in some way to treatment with Glucocorticoids (15). Every decision to start or intensify glucocorticoid therapy needs to be analyzed carefully. Patients should be encouraged to use nonsteroidal anti-inflammatory drugs or antimalarial agents for relatively benign symptoms (16,17).
Acknowledgement
Authors would like to thank Dr. Rachna Kapoor for her help in the preparation of this case report.
References :
  1. Kaslow RA, Masi AT. Age, sex, and race effects on mortality from systemic lupus erythematosus in the United States . Arthritis Rheum 1978; 21 (4): 473-479.
  2. Caeiro F, Michielson FM, Bernstein R, Hughes GR, Ansell BM. Systemic lupus erythematosus in childhood. Ann Rheum Dis 1981; 40: 325-31.
  3. Tucker LB, Menon S, Schaller JG, Isenberg DA. Adult- and childhood-onset systemic lupus erythematosus: a comparison of onset, clinical features, serology, and outcome. Br J Rheumatol 1995; 34: 866-72.
  4. Tan EM, Cohen AS, Fries JF et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1982; 25: 1271-1277.
  5. Marini R, Costallat LT. Young age at onset, renal involvement, and arterial hypertension are of adverse prognostic significance in juvenile systemic lupus erythematosus. Rev Rhum Engl Ed 1999; 66: 303-309.
  6. Pistiner M, Wallace DJ, Nessim S, Metzger AL, Klinenberg JR. Lupus erythematosus in the 1980s: a survey of 570 patients. Semin Arthritis Rheum 1991; 21: 55 -64.
  7. Iverson GL. Psychopathology associated with systemic lupus erythematosus: a methodological review. Semin Arthritis Rheum 1993; 22: 242-251.
  8. Wysenbeek AJ, Guedj D, Amit M, Weinberger A. Rash in systemic lupus erythematosus: prevalence and relation to cutaneous and non-cutaneous disease manifestations. Ann Rheum Dis 1992; 51: 717-719.
  9. Callen JP. Mucocutaneous changes in patients with lupus erythematosus: the relationship of these lesions to systemic disease. Rheum Dis Clin North Am 1988; 14: 79-97.
  10. Sontheimer RD. Subacute cutaneous lupus erythematosus: a decade's perspective. Med Clin North Am 1989; 73: 1073-1090.
  11. Burge SM, Frith PA, Juniper RP, Wojnarowska F. Mucosal involvement in systemic and chronic cutaneous lupus erythematosus. Br J Dermatol 1989; 121: 727-741.
  12. Shergy WJ, Kredich DW, Pisetsky DS. Patterns of autoantibody expression in pediatric and adult systemic lupus erythematosus. J Rheumatol 1989; 16: 1329-1334.
  13. Sibley JT, Olszynski WP, Decoteau WE, Sundaram MG. The incidence and prognosis of central nervous system disease in systemic lupus erythematosus. J Rheumatol 1991; 19: 47 -52.
  14. Quintero-Del-Rio AI, Van Miller. Neurologic symptoms in children with systemic lupus erythematosus. J Child Neurol 2000; 15: 803-7.
  15. Petri M, Spence D, Bone LR, Hochberg MC. Coronary artery disease risk factors in the Johns Hopkins Lupus Cohort: prevalence, recognition by patients, and preventive practices. Medicine ( Baltimore ) 1992; 71: 291-302.
  16. Lockshin MD. Therapy for systemic lupus erythematosus. N Engl J Med 1991; 324: 189- 191.
  17. Klippel JH. Is aggressive therapy effective in lupus - Rheum Dis Clin North Am 1993; 19: 249-261.
Last Updated : Sunday, April 01, 2007 Vol 4 Issue 4 Art #14
How to Cite URL :
Thapar K, Dhawan G. THROMBOCYTOPENIA AS A PRESENTING FEATURE OF SYSTEMIC LUPUS ERYTHEMATOSUS (SLE) . Pediatric Oncall [serial online] 2007[cited 2007 April 1];4. Art #14. Available From : http://www.pediatriconcall.com/Journal/Article/FullText.aspx?artid=153&type=J&tid=&imgid=&reportid=151&tbltype=
Disclaimer: The information given by www.pediatriconcall.com is provided by medical and paramedical & Health providers voluntarily for display & is meant only for informational purpose. The site does not guarantee the accuracy or authenticity of the information. Use of any information is solely at the user's own risk. The appearance of advertisement or product information in the various section in the website does not constitute an endorsement or approval by Pediatric Oncall of the quality or value of the said product or of claims made by its manufacturer.