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Adult-Onset Still Disease Presenting With Dermatomyositis-Like Persistent Pruritic Lesions

Qiao, Jianjun MD, PhD*; Pan, Yunlei MD*; Li, Sheng MD*; Tang, Shunli MD*; Zheng, Siting MD*; Zhu, Dingxian MD*; Sun, Chuanyin MD; Fang, Hong MD*

The American Journal of Dermatopathology: November 2019 - Volume 41 - Issue 11 - p 851–854
doi: 10.1097/DAD.0000000000001447
Extraordinary Case Report

Abstract: Adult-onset Still disease (AOSD) is a rare autoinflammatory condition. The presence of an evanescent, salmon-pink, nonpruritic rash is one of the major diagnostic criteria for the disease. The rash occurs with fever and subsides with defervescence. The presence of dyskeratotic keratinocytes in the upper one-third layer of the epidermis is a distinctive histopathological feature of persistent pruritic lesions associated with AOSD. Here, we report 2 cases of AOSD characterized by persistent pruritic lesions resembling those observed in dermatomyositis. Identifying the clinical and histopathological manifestation of the cutaneous lesions is essential for the early diagnosis of AOSD and for differentiating this condition from those presenting with dyskeratotic cells in the epidermis.

Departments of *Dermatology; and

Rheumatology, The First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, China.

Correspondence: Hong Fang, MD, Department of Dermatology, The First Affiliated Hospital, College of Medicine, Zhejiang University, No. 79, Qingchun Road, Hangzhou 310003, Zhejiang Province, China (e-mail:

Supported by the National Natural Science Foundation of China (Grant no. 81271743 and 81571969).

The authors declare no conflicts of interest.

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