Persistent Pruritic Skin Lesions with Dyskeratotic Cells in Upper Layer of Epidermis Are Specific and Associated with High Levels of Serum IL-18 in Adult-Onset Still’s Disease

Natsuki Maeda^1,2, Yoshinori Taniguchi³, Kimiko Nakajima⁴, Yoshiko Shimamura⁵, Hirofumi Nishikawa⁶, Shuichi Nakayama⁴, Shigetoshi Sano⁴, Shimpei Fujimoto⁶ and Yoshio Terada⁵, ¹Endocrinology,Metabolism,Nephrology and Rheumatology, Kochi Medical School, Nankoku, Japan, ²Dermatology, Kochi Medical School, Nankoku, Japan, ³Endocrinology, Metabolism,Nephrology and Rheumatology, Kochi University, Kochi, Japan, ⁴Kochi Medical School, Nankoku, Japan, ⁵Kochi University, Nankoku, Japan, ⁶Endocrinology, Metabolism, Nephrology and Rheumatology, Kochi Medical School, Nankoku, Japan

Meeting: 2016 ACR/ARHP Annual Meeting

Date of first publication: September 28, 2016

Keywords: adult-onset Still's disease and apoptosis, IL-1/IL-18

Session Information

Date: Sunday, November 13, 2016

Session Title: Miscellaneous Rheumatic and Inflammatory Diseases - Poster I

Session Type: ACR Poster Session A

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

Background/Purpose: Adult-onset Still’s disease (AOSD) is an acute and systemic inflammatory disorder that is characterized by high spiking fever, evanescent rash, arthralgia/arthritis and hyperferritinemia. However, recent reports showed that not only typical evanescent salmon-colored rash but also atypical skin lesions, persistent pruritic papules and plaques, could be associated with AOSD. The atypical skin lesions are histologically characterized to have the dyskeratotic cells in upper layer of epidermis.

Methods: We retrospectively assessed clinical and histological findings of skin lesions including persistent pruritic skin lesions in Japanese patients with AOSD (n=7). Moreover, we compared serological and histological finding of AOSD with that of dermatomyositis (DM) (n=6), drug eruptions (DE) (n=6), and Graft versus Host disease (GVHD) (n=6).

Results: AOSD with persistent pruritic skin lesions (n=5) histologically showed dyskeratotic cells only in upper layer of epidermis and horny layer without intraepidermal infiltrations of inflammatory cells. These dyskeratotic cells were positive by TUNEL and ssDNA stainings, suggesting apoptotic cells. AOSD with evanescent rash (n=2) histologically showed no dyskeratosis. On the other side, the pathological findings of DM (n=6), DE (n=6) and GVHD (n=6) had dyskeratotic cells in all layers of epidermis with inflammatory cells infiltrations. Notably, all of AOSD with atypical skin lesions (n=5) had very high levels of serum IL-18 (74,300~307,000 pg/ml).

Conclusion: AOSD with persistent pruritic skin lesions is characterized and specific by prominent epidermal apoptosis, especially involving the upper layers. Therefore, it could play a pivotal role to recognize the atypical skin lesions of AOSD for correct early diagnosis. Finally, the high levels of serum IL-18 might be related with epidermal apoptosis of keratinocyte in AOSD.

Disclosure: N. Maeda, None; Y. Taniguchi, None; K. Nakajima, None; Y. Shimamura, None; H. Nishikawa, None; S. Nakayama, None; S. Sano, None; S. Fujimoto, None; Y. Terada, None.

To cite this abstract in AMA style:

Maeda N, Taniguchi Y, Nakajima K, Shimamura Y, Nishikawa H, Nakayama S, Sano S, Fujimoto S, Terada Y. Persistent Pruritic Skin Lesions with Dyskeratotic Cells in Upper Layer of Epidermis Are Specific and Associated with High Levels of Serum IL-18 in Adult-Onset Still’s Disease [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/persistent-pruritic-skin-lesions-with-dyskeratotic-cells-in-upper-layer-of-epidermis-are-specific-and-associated-with-high-levels-of-serum-il-18-in-adult-onset-stills-disease/. Accessed January 24, 2021.

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