Adult-Onset Still’s Disease Presenting as an Atypical Cutaneous Eruption

Main Article Content

Cory Kosche
Lida Zheng
Lauren Guggina

Keywords

AOSD, eruption, atypical, adult-onset Still's disease, infliximab

Abstract

Adult-onset Still’s disease classically presents with high fevers, arthralgias, leukocytosis, and an evanescent eruption. There are, however, a known subset of patients who develop an atypical eruption with persistent erythematous to violaceous papules and plaques. Here, we present the case of a white female in her 40s who presented with 2 years of spiking fevers, arthralgias, and a fixed pruritic eruption with erythematous plaques with overlying scale and linear accentuation. She was initially treated with oral prednisone, anakinra, and methotrexate. Due to persistent symptoms, she was switched to adalimumab with significant relief of symptoms. Prompt recognition of adult-onset Still’s disease with this atypical eruption may help prevent delayed or missed diagnosis and allow for early, appropriate intervention.

References

1. Bywaters EG. Still's disease in the adult. Annals of the rheumatic diseases. 1971;30(2):121-133.

2. Efthimiou P, Paik PK, Bielory L. Diagnosis and management of adult onset Still’s disease. Annals of the rheumatic diseases. 2006;65(5):564-572.

3. Yamaguchi M, Ohta A, Tsunematsu T, et al. Preliminary criteria for classification of adult Still's disease. The Journal of rheumatology. 1992;19(3):424-430.

4. Fautreal B, Zing E, Golmard J-L, et al. Proposal for a New Set of Classification Criteria for Adult-Onset Still Disease. Medicine. 2002;81(3):194-200.

5. Kawaguchi Y, Terajima H, Harigai M, Hara M, Kamatani N. Interleukin-18 as a novel diagnostic marker and indicator of disease severity in adult-onset Still's disease. Arthritis & Rheumatism. 2001;44(7):1716-1717.

6. Qiao J, Zhou S, Li S, et al. Histopathological diagnosis of persistent pruritic eruptions associated with adult-onset Still's disease. Histopathology.0(0).

7. Callen JP. Dermatomyositis. The Lancet. 2000;355(9197):53-57.

8. Sun NZ, Brezinski EA, Berliner J, et al. Updates in adult-onset Still disease: Atypical cutaneous manifestations and associations with delayed malignancy. Journal of the American Academy of Dermatology. 2015;73(2):294-303.