Dyskeratotic Cells in Persistent Pruritic Skin Lesions Are Apoptotic and Associated with High Levels of Serum IL-18, and Possibly Predict the Outcomes in Adult-Onset Still’s Disease

Hirofumi Nishikawa¹, Yoshinori Taniguchi¹, Natsuki Maeda¹, Satoshi Inotani¹, Kimiko Nakajima², Kaoru Arii³ and Yoshio Terada¹, ¹Endocrinology, Metabolism, Nephrology and Rheumatology, Kochi Medical School Hospital, Nankoku, Japan, ²Dermatology, Kochi Medical School, Nankoku, Japan, ³Internal Medicine, Kochi Red Cross Hospital, Kochi, Japan

Meeting: 2018 ACR/ARHP Annual Meeting

Keywords: IL-1/IL-18 and Still's disease

Session Information

Date: Monday, October 22, 2018

Title: Miscellaneous Rheumatic and Inflammatory Diseases Poster II: Interstitial Lung Disease, Still's Disease, FMF, Polychondritis

Session Type: ACR Poster Session B

Session Time: 9:00AM-11:00AM

Background/Purpose: To assess the clinical significance of dyskeratotic cells (DCs) in skin lesions of adult-onset Still’s disease (AOSD).

Methods: We assessed clinical characteristics, serum markers, outcomes and histology of skin lesions in Japanese patients with AOSD (n=15). Moreover, we comparatively studied the histological findings of AOSD, dermatomyositis (DM) (n=6), drug eruptions (DE) (n=7), and graft versus host disease (GVHD) (n=6).

Results: AOSD with persistent pruritic skin lesions (n=10) histologically showed DCs only in upper layer of epidermis and horny layer without inflammatory cells infiltrations, indicating dyskeratosis. AOSD with evanescent rash (n=5) histologically showed no DCs. In contrast, the histology of DM, DE and GVHD demonstrated that DCs existed in all layers of epidermis with inflammatory cells infiltrations. DCs in AOSD were positive by ssDNA staining, suggesting apoptotic cells. Serum IL-18 showed significantly higher in AOSD patients with DCs (n=10) than without DCs (n=5). The majority of AOSD patients with DCs required higher doses of glucocorticoids, immunosuppressants and biologic agents than without DCs. 2 of 10 AOSD patients with DCs died because of infectious complications or hemophagocytic syndrome.

Conclusion: Persistent pruritic skin lesions in AOSD are specific by prominent epidermal apoptosis involving the upper layers of epidermis, and hyper IL-18 is significantly related with dyskeratosis. The appearance of DCs in AOSD seems to be associated with poor prognosis.

Disclosure: H. Nishikawa, None; Y. Taniguchi, None; N. Maeda, None; S. Inotani, None; K. Nakajima, None; K. Arii, None; Y. Terada, None.

To cite this abstract in AMA style:

Nishikawa H, Taniguchi Y, Maeda N, Inotani S, Nakajima K, Arii K, Terada Y. Dyskeratotic Cells in Persistent Pruritic Skin Lesions Are Apoptotic and Associated with High Levels of Serum IL-18, and Possibly Predict the Outcomes in Adult-Onset Still’s Disease [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/dyskeratotic-cells-in-persistent-pruritic-skin-lesions-are-apoptotic-and-associated-with-high-levels-of-serum-il-18-and-possibly-predict-the-outcomes-in-adult-onset-stills-disease/. Accessed .

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