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Case Reports in Rheumatology
Volume 2016 (2016), Article ID 4835147, 4 pages
http://dx.doi.org/10.1155/2016/4835147
Case Report

Atypical Cutaneous Manifestations in Adult Onset Still’s Disease

1Barwon Health, Bellerine Street, Geelong, VIC 3220, Australia
2Barwon Rheumatology Service, 156 Bellerine Street, Geelong, VIC 3220, Australia

Received 8 November 2015; Revised 18 January 2016; Accepted 19 January 2016

Academic Editor: Tsai-Ching Hsu

Copyright © 2016 Champa Nataraja and Hedley Griffiths. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Adult Onset Still’s Disease (AOSD), an adult variant of systemic onset juvenile idiopathic arthritis, is a rare systemic inflammatory disorder of unknown aetiology. The rarity of this disease is associated with low index of suspicion and delayed diagnosis in patients suffering from it and in the presence of atypical features the diagnosis can be further challenging. This is a case report on a 24-year-old woman, who was a diagnostic dilemma for 2 years due to the nonspecific symptoms of recurrent fever, generalized maculopapular persistent pruritic and tender rash, and polyarthralgia. She was initially diagnosed as leukocytoclastic vasculitis on a skin biopsy and was managed by a dermatologist with various medications including NSAIDs, hydroxychloroquine, dapsone, colchicine, cyclosporine, and high doses of oral steroids with minimal response. Subsequently, she has had multiple admissions with similar symptoms with raised inflammatory markers and negative septic workup. On one occasion, her iron study revealed hyperferritinaemia which led to the suspicion of AOSD. Once the rheumatic fever and infectious, malignant, autoimmune, and lymphoproliferative disorders were excluded, she was diagnosed as probable AOSD and managed successfully with IL-1 (interleukin-1) receptor antagonist, Anakinra, with remarkable and lasting response both clinically and biochemically.