Neutrophilic myositis developing in a case of systemic lupus erythematosus
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8. Takahashi T, Asano Y, Sugawara K, et al. Epithelial Fli1 deficiency drives systemic autoimmunity and fibrosis: possible roles in scleroderma. J Exp Med 2017; 214: 1129-51. 9. Distler JH, Jüngel A, Pileckyte M, et al. Hypoxia-induced increase in the production of extracellular matrix proteins in systemic sclerosis. Arthritis Rheum 2007; 56: 4203-15. 10. Sambo P, Baroni SS, Luchetti M, et al. Oxidative stress in scleroderma: maintenance of scleroderma fibroblast phenotype by the constitutive up-regulation of reactive oxygen species generation through the NADPH oxidase complex pathway. Arthritis Rheum 2001; 44: 2653-64. doi:10.1684/ejd.2020.3808
These clinical and histological findings suggest that LSclike lesions are induced by the Köbner phenomenon in some ACA-positive SSc patients. Supporting this notion, Fli1flox/flox ; K14Cre ; Rag1-/- mice, which reproduce the phenotype of SSc keratinocytes such as excessive IL-1␣ production and lack mature B cells and T cells, display skin fibrosis accompanied by an infiltration and degranulation of mast cells [8]. Alternatively, local compression may induce vascular injury, leading to the activation of SSc dermal fibroblasts through tissue hypoxia and oxidative stress [9, 10]. Our patient was positive for ACA and developed uniquely distributed skin lesions on the areas exposed to local compression related to playing volleyball. Importantly, her LSc-like lesions gradually resolved after she stopped playing volleyball despite no change in other parts of affected skin, suggesting that LSc-like lesions are reversible. In summary, our SSc patient presented with LSc-like lesions with unique presentation and clinical course, suggesting that this type of skin lesion can be treated by avoiding mechanical stress. Dermatologists should be aware of this clinical entity. Disclosures. Financial support: none. Conflicts of interest: none. Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo 113-8655, Japan
Yoshihide ASANO Shinichi SATO
1. Tuffanelli DL, Winkelmann RK. Systemic scleroderma, a clinical study of 727 cases. Arch Dermatol 1961; 84: 359-71. 2. Asano Y, Fujimoto M, Ishikawa O, et al. Diagnostic criteria, severity classification and guidelines of localized scleroderma. J Dermatol 2018; 45: 755-80. 3. Miura S, Someya M, Toyama S, et al. A case of scleroderma en coup de sabre with ipsilateral hearing loss and aphakia. Eur J Dermatol 2019; 29: 423-5. 4. Takahashi T, Asano Y, Oka T, et al. Scleroderma en coup de sabre with recurrent episodes of brain hemorrhage. J Dermatol 2016; 43: 203-6. 5. Happle R. Superimposed segmental manifestation of polygenic skin disorders. J Am Acad Dermatol 2007; 57: 690-9. 6. Saigusa R, Asano Y, Yamashita T, et al. Systemic sclerosis complicated with localized scleroderma-like lesions induced by Köbner phenomenon. J Dermatol Sci 2018; 89: 282-9. 7. Ehara M, Oono T, Yamasaki O, Matsuura H, Iwatsuki K. Generalized morphea-like lesions arising in mechanically-compressed areas by underclothes. Eur J Dermatol 2006; 16: 307-9.
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