Updates in adult-onset Still disease: Atypical cutaneous manifestations and associations with delayed malignancy - 15/07/15

Doi : 10.1016/j.jaad.2015.04.063

Natalie Z. Sun, MD ^a,^⁎^{^{∗
Drs Sun and Brezinski contributed equally to this work.}} , Elizabeth A. Brezinski, MD ^b,^{^{∗
Drs Sun and Brezinski contributed equally to this work.}}, Jacqueline Berliner, MD ^a, Anna Haemel, MD ^a, M. Kari Connolly, MD ^a, Lianne Gensler, MD ^c, Timothy H. McCalmont, MD ^a,^d, Kanade Shinkai, MD, PhD ^a
^a Department of Dermatology, University of California San Francisco, San Francisco, California
^c Department of Rheumatology, University of California San Francisco, San Francisco, California
^d Department of Pathology, University of California San Francisco, San Francisco, California
^b Department of Internal Medicine, California Pacific Medical Center, San Francisco, California

^∗Reprint requests: Natalie Z. Sun, MD, Department of Dermatology, University of California San Francisco, 1701 Divisadero St, 3rd fl, San Francisco, CA 94115.

Abstract

Adult-onset Still disease (AOSD) is a systemic inflammatory disorder that is clinically characterized by a heterogeneous constellation of symptoms and signs. Though an evanescent eruption is the classic cutaneous finding, recent literature has highlighted atypical rashes associated with Still disease. A second emerging concept in presentations of AOSD is its association with malignancy. This review focuses on these concepts: the clinical spectrum of atypical skin manifestations and AOSD as a paraneoplastic phenomenon. PubMed-MEDLINE was screened for peer-reviewed articles describing atypical presentations of AOSD and cases associated with malignancy. Erythematous, brown or violaceous, persistent papules and plaques were the most common cutaneous finding (28/30 [93%]). Linear configurations were also rarely described. Of these patients, 81% concurrently had the typical evanescent skin eruption. There were 31 patients with associated malignancies, most commonly breast cancer and lymphoma. The diagnosis of malignancy did not precede or immediately follow a clinical presentation otherwise consistent with AOSD in a considerable subset of patients (42%). Understanding the cutaneous spectrum of AOSD and heightened awareness for its delayed association with malignancy may lead to improved recognition of cutaneous variants and reinforce the need for diagnostic evaluation and long-term follow-up for malignancy in patients with this clinical presentation.

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Key words : adult-onset Still disease, evanescent rash, hyperferritinemia, paraneoplastic syndrome, persistent pruritic eruption

Abbreviations used : ANA, AOSD, GF