title: An unusual presentation of juvenile systemic lupus erythematosus.
abstract: Systemic lupus erythematosus (SLE) is a multisystem disorder with a diverse spectrum of clinical abnormality. Despite advances in our understanding of the disease, the aetiology and outcome remains unknown. We describe a 13-year-old boy who presented with paronychia, mouth ulcers and associated neutropenia. Appropriate investigations including a bone-marrow biopsy revealed a shift to the left, and in the absence of any other immunological abnormalities, a diagnosis of idiopathic neutropenia was made. The patient was successfully treated with granulocyte colony-stimulating factor but subsequently re-presented during the winter months with a purpuric facial eruption affecting his cheeks and ears. On histological examination of a biopsy taken from the ear, a microvascular thrombosis was seen. Laboratory investigations were positive for anticardiolipin antibodies, neutropenia and antinuclear antibodies (ANA), suggesting a diagnosis of juvenile (J)JSLE with antiphospholipid syndrome. This is an unusual presentation of SLE in a prepubertal white boy, with a malar and helical vasculopathy as a novel cutaneous presentation of JSLE.
authors.affiliation: Department of Dermatology, Queens Medical Centre, Nottingham University Hospitals NHS Trust, Nottingham, UK. dranandpatel@doctors.net.uk
authors.forename: A
authors.initials: A
authors.lastname: Patel
authors.forename: S
authors.initials: S
authors.lastname: Rangaraj
authors.forename: B
authors.initials: B
authors.lastname: Wright
authors.forename: R
authors.initials: R
authors.lastname: Murphy
mesh_terms: D000293:Adolescent; D016736:Antiphospholipid Syndrome / Q000175:diagnosis; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000175:diagnosis; D008297:Male; D009503:Neutropenia / Q000209:etiology*; D019226:Oral Ulcer / Q000209:etiology*; D010304:Paronychia / Q000209:etiology*; D016896:Treatment Outcome