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368 Immune thrombocytopenia associated with localisedlocalized scleroderma- report of 4 paediatricpediatriccases
  1. A Jindal1,
  2. A Gupta1,
  3. S Dogra2,
  4. A Rawat1,
  5. D Suri1,
  6. J Ahluwalia3 and
  7. S Singh1
  1. 1PGIMER, Paediatrics, chandigarh, India
  2. 2PGIMER, Dermatology, chandigarh, India
  3. 3PGIMER, Haematology, chandigarh, India

Abstract

Background and aims Many autoimmune and inflammatory diseases have been found to be associated with morphea. Thrombocytopenia, however, has rarely been reported

Aim-To describe the profile of 4 patients with localised scleroderma and thrombocytopenia

Methods A retrospective case record review of children diagnosed to have localised scleroderma in the paediatric rheumatology clinic of a tertiary care referral institute in North-West India. Children who also had thrombocytopenia (i.e. platelet counts <150 ×103/L) were analysed.

Results Twenty two children were diagnosed with localised scleroderma (10 boys and 12 girls, male: female ratio 1:1.2). Thrombocytopenia was identified in 4 children (prevalence-18%). The details of these 4 cases are summarised in Table 1. All 4 cases had en coup de sabre (ECDS) type of linear scleroderma. (Figure 1) Peripheral smear examination revealed normal platelet size. The lowest platelet counts ranged from 8×109/L to 120×109/L. ANA was positive in 2 patients (case 1 and 2) and case 1 also had positive lupus anticoagulant in her serum. Methotrexate (0.5 mg/kg/week) was used for the treatment of morphea in 3 patients while one child was treated with topical calcipotriol ointment alone. No specific therapy was prescribed for thrombocytopenia, except in case 1 where thrombocytopenia was treated with injection anti D. Platelet counts recovered in 3 patients, while thrombocytopenia is still persisting in one patient.

Abstract 368 Table 1
Abstract 368 Table 1
Abstract 368 Figure 1
Abstract 368 Figure 1

Conclusions Thrombocytopenia associated with localised scleroderma in children is usually benign, has a probable autoimmune aetiology, requires no specific therapy and is generally curable with the systemic immunosuppressant medications used for the treatment of scleroderma

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