Association Between Peripheral Eosinophilia and Clinical Characteristics of Adult-onset Still’s Disease with Persistent Eruption: A Retrospective Study

Authors

  • Jia-Wei Liu
  • Tian Chen
  • Wei Liu
  • Ru-Xuan Chen
  • Yue-Tong Qian
  • Yan Tan
  • Dong-Lai Ma Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases, 100730 Beijing, China

DOI:

https://doi.org/10.2340/00015555-3927

Keywords:

persistent eruption, adult-onset Still’s disease, eosinophilia, body surface area, clinical characteristics

Abstract

Persistent eruption occurs in a subset of patients with adult-onset Still’s disease. In our experience, a considerable proportion of these patients present with peripheral eosinophilia. The aims of this study were to summarize the clinical and histological features of patients with adult-onset Still’s disease with persistent eruption in the current study cohort, and to assess the association between peripheral eosinophil levels and disease characteristics. A total of 21 patients with adult-onset Still’s disease with persistent eruption were included in this retrospective study. Koebner signs, an important diagnostic clue, were found in 85.7% of patients. The proportion of patients presenting with eosinophilia within the disease course was 57.1%. Skin histology revealed infiltration of eosinophils in 90.5% of patients. Peripheral eosinophil levels were positively associated with involved body surface area. Patients with normal peripheral eosinophil counts were more likely to achieve complete remission than those with abnormal peripheral eosinophil counts. Eosinophils may be involved in the pathogenesis of skin eruption. Abnormal peripheral eosinophil counts in these patients may indicate a more refractory disease course.

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References

Bywaters EG. Still’s disease in the adult. Ann Rheum Dis 1971; 30: 121–133.

Fautrel B, Le Moël G, Saint-Marcoux B, Taupin P, Vignes S, Rozenberg S, et al. Diagnostic value of ferritin and glycosylated ferritin in adult onset Still’s disease. J Rheumatol 2001; 28: 322–329.

Uppal SS, Al-Mutairi M, Hayat S, Abraham M, Malaviya A. Ten years of clinical experience with adult onset Still’s disease: is the outcome improving? Clin Rheumatol 2007; 26: 1055–1060.

Yamamoto T. Cutaneous manifestations associated with adult-onset Still’s disease: important diagnostic values. Rheumatol Int 2012; 32: 2233–2237.

Suzuki K, Kimura Y, Aoki M, Takezaki S, Tuchida T, Takano T, et al. Persistent plaques and linear pigmentation in adult-onset Still’s disease. Dermatology 2001; 202: 333–335.

Lee JY, Hsu CK, Liu MF, Chao SC. Evanescent and persistent pruritic eruptions of adult-onset still disease: a clinical and pathologic study of 36 patients. Semin Arthritis Rheum 2012; 42: 317–326.

Maeda-Aoyama N, Hamada-Ode K, Taniguchi Y, Nishikawa H, Arii K, Nakajima K, et al. Dyskeratotic cells in persistent pruritic skin lesions as a prognostic factor in adult-onset Still disease. Medicine (Baltimore) 2020; 99: e19051.

Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H, et al. Preliminary criteria for classification of adult Still’s disease. J Rheumatol 1992; 19: 424–430.

Efthimiou P, Paik PK, Bielory L. Diagnosis and management of adult onset Still’s disease. Ann Rheum Dis 2006; 65: 564–572.

Ohta A, Yamaguchi M, Tsunematsu T, Kasukawa R, Mizushima H, Kashiwagi H, et al. Adult Still’s disease: a multicenter survey of Japanese patients. J Rheumatol 1990; 17: 1058–1063.

Kaur S, Bambery P, Dhar S. Persistent dermal plaque lesions in adult onset Still’s disease. Dermatology 1994; 188: 241–242.

Criado RF, Criado PR, Vasconcellos C, Szajubok JC, Michalany NS, Kadunc BV, et al. Urticaria as a cutaneous sign of adult-onset Still’s disease. J Cutan Med Surg 2006; 10: 99–103.

Soy M. A case of adult-onset Still’s disease presenting with angioedema. Clin Rheumatol 2004; 23: 92.

Lee JB, Kim JW, Lee SS, Kim SJ, Won YH, Lee SC. Adult-onset Still’s disease with vesiculopustules on the hands and feet. J Korean Med Sci 2002; 17: 852–855.

Fujii K, Konishi K, Kanno Y, Ohgou N. Persistent generalized erythema in adult-onset Still’s disease. Int J Dermatol 2003; 42: 824–825.

Lee JY, Yang CC, Hsu MM. Histopathology of persistent papules and plaques in adult-onset Still’s disease. J Am Acad Dermatol 2005; 52: 1003–1008.

Qiao J, Zhou S, Li S, Xue R, Pan Y, Tang S, et al. Histopathological diagnosis of persistent pruritic eruptions associated with adult-onset Still’s disease. Histopathology 2019; 74: 759–765.

Yamamoto T, Nishioka K. Flagellate erythema. Int J Dermatol 2006; 45: 627–631.

Peckruhn M, Elsner P, Tittelbach J. Eosinophilic dermatoses. J Dtsch Dermatol Ges 2019; 17: 1039–1051.

Kuang FL. Approach to patients with eosinophilia. Med Clin North Am 2020; 104: 1–14.

Park SH, Lee SH, Kim JH, Kim SC. Circulating eosinophil and neutrophil counts correlate with disease severity in bullous pemphigoid. Ann Dermatol 2018; 30: 544–549.

Simon D, Braathen LR, Simon HU. Eosinophils and atopic dermatitis. Allergy 2004; 59: 561–570.

Gerfaud-Valentin M, Jamilloux Y, Iwaz J, Sève P. Adult-onset Still’s disease. Autoimmun Rev 2014; 13: 708–722.

Sanders NL, Mishra A. Role of interleukin-18 in the pathophysiology of allergic diseases. Cytokine Growth Factor Rev 2016; 32: 31–39.

Han MW, Kim SH, Oh I, Kim YH, Lee J. Serum IL-1β can be a biomarker in children with severe persistent allergic rhinitis. Allergy Asthma Clin Immunol 2019; 15: 58.

Walsh SA, Creamer D. Drug reaction with eosinophilia and systemic symptoms (DRESS): a clinical update and review of current thinking. Clin Exp Dermatol 2011; 36: 6–11.

Böer A, Ackerman AB. Prurigo pigmentosa is distinctive histopathologically. Int J Dermatol 2003; 42: 417–418.

Mitrovic S, Fautrel B. Complications of adult-onset Still’s disease and their management. Expert Rev Clin Immunol 2018; 14: 351–365.

Arlet JB, Le TH, Marinho A, Amoura Z, Wechsler B, Papo T, et al. Reactive haemophagocytic syndrome in adult-onset Still’s disease: a report of six patients and a review of the literature. Ann Rheum Dis 2006; 65: 1596–1601.

Sfriso P, Priori R, Valesini G, Rossi S, Montecucco CM, D’Ascanio A, et al. Adult-onset Still’s disease: an Italian multicentre retrospective observational study of manifestations and treatments in 245 patients. Clin Rheumatol 2016; 35: 1683–1689.

Narváez J, Mora-Limiñana M, Ros I, Ibañez M, Valldeperas J, Crémer D, et al. Pulmonary arterial hypertension in adult-onset Still’s disease: a case series and systematic review of the literature. Semin Arthritis Rheum 2019; 49: 162–170.

Published

2021-09-28

How to Cite

Liu, J.-W., Chen, T., Liu, W., Chen, R.-X., Qian, Y.-T., Tan, Y., & Ma, D.-L. (2021). Association Between Peripheral Eosinophilia and Clinical Characteristics of Adult-onset Still’s Disease with Persistent Eruption: A Retrospective Study. Acta Dermato-Venereologica, 101(9), adv00564. https://doi.org/10.2340/00015555-3927