Kikuchi Nobuyuki, Satoh Masataka, Ohtsuka Mikio, Yamamoto Toshiyuki
Department of Dermatology, Fukushima Medical University, Fukushima, Japan.
J Dermatol. 2014 May;41(5):407-10. doi: 10.1111/1346-8138.12426. Epub 2014 Mar 15.
Typical skin rash, which appears and disappears along with respective rise and fall of fever, is well-known, and included as one of the major criteria of adult-onset Still's disease (AOSD) (Yamaguchi's criteria). In addition, various skin lesions are occasionally observed in association with AOSD. Persistent pruritic eruptions present with some clinical features, such as urticarial erythema, flagellate erythema, erythematous, slightly scaly or crusted papules, and/or plaques on the trunk and extremities. These lesions show unique histological features such as dyskeratosis with a peculiar, distinctive distribution in the upper epidermis and cornified layers with focal hyperkeratosis. We describe herein six cases of AOSD, which presented with skin lesions of persistent pruritic papules and plaques. All six cases were female, and three of them were elderly women. The patients presented with linear erythematous streaks, scaly erythema, keratotic papules, infiltrative plaques and irregular coalesced erythemas. By contrast, histological features were characteristic, and dyskeratotic cells were found in the horny layers as well as in the upper layers of the epidermis. Persistent pruritic eruption is an important cutaneous sign for the diagnosis of AOSD.
典型的皮疹会随着发热的相应起伏而出现和消退，这是众所周知的，并且被纳入成人斯蒂尔病（AOSD）的主要标准之一（山口标准）。此外，AOSD偶尔还会出现各种皮肤病变。持续性瘙痒性皮疹具有一些临床特征，如躯干和四肢出现风团样红斑、鞭状红斑、红斑性、轻度鳞屑或结痂丘疹和/或斑块。这些病变具有独特的组织学特征，如在表皮上层和角质层有角化不良，且分布特殊，伴有局灶性角化过度。我们在此描述6例AOSD病例，其表现为持续性瘙痒性丘疹和斑块的皮肤病变。所有6例均为女性，其中3例为老年女性。患者表现为线状红斑条纹、鳞屑性红斑、角化性丘疹、浸润性斑块和不规则融合性红斑。相比之下，组织学特征具有特异性，在角质层以及表皮上层发现角化不良细胞。持续性瘙痒性皮疹是诊断AOSD的重要皮肤体征。
