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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.
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