DOI: 10.12890/2025_005387

Adult-onset Still’s disease with dermatomyositis-like lesions

Authors

  • Inés Segovia Rodríguez Department of Dermatology, Infanta Cristina University Hospital, Madrid, Spain
  • Juan Vicente de la Sota Internal Medicine Department, Infanta Cristina University Hospital, Madrid, Spain
  • Alba Hernández Piriz Internal Medicine Department, Infanta Cristina University Hospital, Madrid, Spain
  • María Castillo Gutiérrez Department of Dermatology, Infanta Cristina University Hospital, Madrid, Spain
  • Teresa López Bernal Department of Dermatology, Infanta Cristina University Hospital, Madrid, Spain
  • Beatriz Aranegui Arteaga Department of Dermatology, Infanta Cristina University Hospital, Madrid, Spain
  • Elena García Guijarro Internal Medicine Department, Infanta Cristina University Hospital, Madrid, Spain

elenag.guijarro@gmail.com

Keywords

Adult-onset Still’s disease, heliotrope rash, dermatomyositis, atypical cutaneous lesions

Abstract

Still’s disease is an inflammatory disorder of unknown origin, also known as juvenile idiopathic arthritis, that predominantly affects children, as it usually appears before the age of 16. However, there is another presentation known as adult-onset Still’s disease, which has a bimodal distribution with the first peak of incidence between 16 and 25 years and the second peak between 36 and 46 years. Classically, it is described as a very typical clinical picture, mainly characterised by a transient salmon-coloured rash that appears with fever spikes, typically in the evening. Additionally, most patients frequently present with symptoms such as a sore throat, generalised lymphadenopathy and hepatosplenomegaly. Less common findings include myopericarditis, interstitial lung disease, serositis and neurological involvement. However, sometimes this disease can debut with more atypical signs and symptoms, delaying diagnosis and treatment.
This article describes the case of a 56-year-old Spanish patient who presented with pruritic periorbital lesions resembling the heliotrope rash of dermatomyositis but was ultimately diagnosed with adult-onset Still’s disease. This case is reported so that in the presence of such cutaneous lesions, Still’s disease is considered within the differential diagnosis to avoid delays in both diagnosis and treatment.

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References

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Published

2025-05-20

How to Cite

1.
Segovia Rodríguez I, Vicente de la Sota J, Hernández Piriz A, Castillo Gutiérrez M, López Bernal T, Aranegui Arteaga B, et al. Adult-onset Still’s disease with dermatomyositis-like lesions. EJCRIM [Internet]. 2025 May 20 [cited 2026 Nov. 3];12(6). Available from: https://ejcrim.com/index.php/ejcrim/article/view/5387