Wolgamot Greg, Yoo Jane, Hurst Stan, Gardner Greg, Olerud John, Argenyi Zsolt
Department of Pathology, University of Washington, Seattle, WA 98195-6100, USA.
Am J Dermatopathol. 2007 Apr;29(2):194-6. doi: 10.1097/DAD.0b013e3180332826.
Adult-onset Still disease (AOSD) is an uncommon disorder characterized by fever, polyarthralgia, elevated white blood cell count, and a maculopapular rash, the histologic features of which have not been well-known. A 55-year-old Asian woman presented initially with a "burning" and severely pruritic eruption on her face, hands, and arms, thought clinically to be urticaria. Within 1 month, she began spiking high fevers, developed diffuse joint pain, and had marked elevations of ferritin, C-reactive protein, and erythrocyte sedimentation rate, characteristic of AOSD. The cutaneous eruption became more widespread, involving the trunk, scalp, and remainder of the extremities, with diffuse thickening of the skin with papular and linear hyperpigmentation and accentuation. Biopsies from several locations showed focal hyperkeratosis associated with dyskeratotic keratinocytes with a peculiar, distinctive distribution in the upper epidermis and cornified layers. In addition, increased dermal mucin was present, with minimal fibroblast proliferation and inflammation. This unusual combination of diffuse dermal mucinosis and a unique pattern of dyskeratosis can present a challenge in generating an accurate differential diagnosis, and may represent an unusual response to chronic scratching or be a distinctive histologic manifestation of AOSD.
成人斯蒂尔病（AOSD）是一种罕见的疾病，其特征为发热、多关节痛、白细胞计数升高和斑丘疹，其组织学特征尚不为人所知。一名55岁的亚洲女性最初表现为面部、手部和手臂出现“烧灼感”且严重瘙痒的皮疹，临床诊断为荨麻疹。在1个月内，她开始出现高热，发展为弥漫性关节疼痛，铁蛋白、C反应蛋白和红细胞沉降率显著升高，这是AOSD的特征。皮肤皮疹变得更加广泛，累及躯干、头皮和四肢其余部位，皮肤弥漫性增厚，伴有丘疹和线状色素沉着及加深。多个部位的活检显示局灶性角化过度，伴有角化异常的角质形成细胞，在上表皮和角质层有特殊、独特的分布。此外，真皮粘蛋白增加，成纤维细胞增殖和炎症轻微。弥漫性真皮粘蛋白病与独特的角化异常模式的这种不寻常组合，在进行准确的鉴别诊断时可能会带来挑战，可能代表对慢性搔抓的异常反应，或者是AOSD的一种独特组织学表现。
