Lee Julia Yu-Yun, Yang Chao-Chun, Hsu Mark Ming-Long
Department of Dermatology, College of Medicine, National Cheng Kung University, Tainan, Taiwan.
J Am Acad Dermatol. 2005 Jun;52(6):1003-8. doi: 10.1016/j.jaad.2005.02.032.
Persistent plaques and linear pigmentation have been reported as specific skin lesions in some patients with adult-onset Still's disease (AOSD).
We sought to characterize the histologic findings of AOSD-associated persistent rash in 11 cases and correlate the histologic findings with the clinical features.
From 1988 to 2004, 17 cases fulfilling Yamaguchi's criteria for AOSD in our hospital were reviewed and 11 (65%) manifested persistent papules and plaques. The pathology of 13 biopsy specimens of persistent eruption from 9 patients was reviewed.
The 11 patients consisted of 3 men and 8 women with age of onset ranging from 19 to 67 years (average 34.7 years). Evanescent Still's rash was recorded in 9 patients. The persistent rash manifested as pruritic, red, violaceous, or brownish scaly or crusted lichenoid papules and plaques usually widely distributed over the trunk, neck, face, and extensor sides of the extremities. Lesions arranged in a bizarre linear pattern resulting from scratching were noted in some patients. Three patients died of severe disease, systemic complications, or both. The histology of persistent papules and plaques was characterized by: (1) multiple individual necrotic keratinocytes, singly or in aggregates, mainly located in the upper epidermis, including the normal or parakeratotic horny layer; and (2) infiltration of lymphocytes and neutrophils in the papillary and middermis. Other less common findings included basal vacuolar alteration, nuclear dust, and subcorneal or intracorneal pustules.
A clinically and pathologically distinct form of persistent lichenoid eruption was commonly observed in our patients with AOSD. The combination of multiple individual necrotic keratinocytes in the upper epidermis and a dermal infiltrate of neutrophils allow for histologic differentiation of this persistent eruption from most other lichenoid and interface dermatitides and may facilitate an earlier diagnosis of AOSD.
在一些成人斯蒂尔病（AOSD）患者中，持续性斑块和线状色素沉着被报道为特异性皮肤损害。
我们试图描述11例AOSD相关持续性皮疹的组织学表现，并将组织学表现与临床特征相关联。
回顾1988年至2004年我院符合山口标准的17例AOSD患者，其中11例（65%）表现为持续性丘疹和斑块。回顾了9例患者13份持续性皮疹活检标本的病理情况。
11例患者中男性3例，女性8例，发病年龄19至67岁（平均34.7岁）。9例患者有一过性斯蒂尔皮疹。持续性皮疹表现为瘙痒性、红色、紫红色或褐色鳞屑或结痂的苔藓样丘疹和斑块，通常广泛分布于躯干、颈部、面部及四肢伸侧。部分患者可见因搔抓形成的奇异线状排列的皮损。3例患者死于严重疾病、全身并发症或两者皆有。持续性丘疹和斑块的组织学特征为：（1）多个单个坏死角质形成细胞，单个或聚集存在，主要位于表皮上层，包括正常或不全角化的角质层；（2）乳头层和真皮中层有淋巴细胞和中性粒细胞浸润。其他较少见的表现包括基底空泡改变、核尘以及角层下或角质层内脓疱。
在我们的AOSD患者中常见一种临床和病理上独特的持续性苔藓样皮疹形式。表皮上层多个单个坏死角质形成细胞与真皮中性粒细胞浸润相结合，有助于将这种持续性皮疹与大多数其他苔藓样和界面性皮炎进行组织学鉴别，可能有助于AOSD的早期诊断。
