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Lookup NU author(s): Dr Katie BestORCiD, Dr Suzy Leech
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A 7-year-old female with type IV skin and autism spectrum disorder presented with a 4 month history of progressive proximal myopathy, weight loss and 20 month history of a worsening, intensely pruritic rash. Cutaneous examination showed a widespread eczematous eruption, post-inflammatory hypopigmentation, heliotrope rash and firm subcutaneous changes, in addition to palmar-plantar papules, pustules and burrows. Extra-cutaneous findings included multiple fixed flexion deformities and muscle wasting. Investigations revealed a normocytic anaemia, elevated muscle enzymes, strongly positive anti-nuclear antibodies and negative myositis-specific antibodies. Whole body MRI reported proximal muscle oedema. Skin biopsy demonstrated lichenoid and perifollicular inflammation, dermal mucin and subcutaneous calcification. Clinical findings were consistent with a diagnosis of juvenile dermatomyositis (JDM), complicated by concurrent scabies.Prednisolone in combination with methotrexate was commenced as first-line therapy in line with current consensus JDM treatment plans, in addition to topical steroids and sun protection. Muscle power started to improve after 5 months but ongoing cutaneous inflammation prompted second-line therapy with IV immunoglobulin. Oral ivermectin was required for the management of scabies due to topical permethrin failure.JDM is a rare autoimmune small-vessel vasculopathy with significant morbidity and limited evidence-based guidelines. Unlike adult-onset disease there is no racial predilection or association with malignancy. Calcinosis is more common in children but early treatment reduces disease complications. JDM has a variable disease course with a median time to remission of 4.67 years. Residual skin changes are increasingly recognised to be an early predictor of prolonged recovery time and these patients may benefit from biologic agents.
Author(s): Best K, Leech S
Publication type: Conference Proceedings (inc. Abstract)
Publication status: Published
Conference Name: 38th Annual Meeting of the British Society for Paediatric and Adolescent Dermatology
Year of Conference: 2023
Pages: i3-i4
Print publication date: 01/01/2024
Online publication date: 03/01/2024
Acceptance date: 02/04/2023
ISSN: 0007-0963
Publisher: Wiley-Blackwell Publishing Ltd.
URL: https://doi.org/10.1093/bjd/ljad483.008
DOI: 10.1093/bjd/ljad483.008
Series Title: British Journal of Dermatology
