P050 Rapidly worsening subcutaneous oedema in a young boy: a case of juvenile dermatomyositis

Abstract

Abstract Background/Aims Juvenile onset dermatomyositis (JDM) is a rare disease with an incidence of 2-4 per million. The presentation includes skin manifestations of heliotrope rash, Gottron's papules, nail fold changes, bilateral peri-orbital or upper eyelid edema and focal mild facial swelling with muscle weakness. Widespread and generalized subcutaneous oedema is a rare presentation in JDM. Methods A 4-year-old previously healthy boy presented to a tertiary care hospital in Sri Lanka with generalized rash involving the face, limbs and trunk for seven days associated with facial puffiness and hair loss. He also complained of generalized myalgia and irritability and refused to walk. He did not have dysphagia, dysphonia or shortness of breath. He had a prodromal febrile illness two weeks back that had been treated as a viral fever. He had evidence of palatal ulcers, chelitis, malar rash, erythema of eyelids, eyelid swelling and necrotic vasculitic lesions in peripheries. He had tenderness over his thighs with muscle power being MRC grade 3/5 in the proximal muscles of his lower limbs. Arm and neck flexors’ power was normal. Results His ESR was raised at 35 mm/hour and CRP was normal. His CPK was 2726 U/l, AST was raised at 931 U/l, ALT was 511 U/l and albumin was 33 g/l. His ANA, dsDNA and ENA panel was negative. A myositis immunoblot could not be done due to financial constraints. An EMG showed evidence of myositis in his proximal muscles. He fulfilled the criteria for juvenile dermatomyositis based on Bohan and Peter’s criteria. He was treated with high dose steroids, dexamethasone 2 mg 6 hourly. While receiving steroids he deteriorated with worsening subcutaneous oedema of face and neck with high fever spikes. The oedema progressed to involve his whole body. His muscle power deteriorated with involvement of his arms. A venogram ruled out any obstructive venous disease. A CT CAP ruled out any underlying malignancy. His 2D echo revealed normal heart function. He was converted to IV Methylprednisolone 30 mg/kg and was started on plasmapharesis. He improved after the 3rd cycle and was started on cyclophosphamide infusions. He received six cyclophosphamide pulses with a tapering course of steroids. He remains controlled in his disease with no new muscle weakness or skin manifestations. However, he remains steroid dependent and is awaiting treatment with Rituximab. Conclusion Subcutaneous oedema in JDM is attributed to a vasculopathy. This is associated with severe rapidly progresssive disease with high mortality and requires aggressive immunosuppression. As seen in our patient disease is resistant to conventional treatment. Early recognition leads to better outcomes in these patients. Disclosure S. Janagan: None. W.A.E. Udeshika: None. P.M. Samarasinghe: None. J.D. Jagoda: None.