与成人Still病相关的皮肤表现

成人Still病是一种病因未明的系统性炎症性疾病,临床上以规律性发热、关节炎或关节痛、淋巴结肿大、肝脾肿大及皮疹为主要特征.现有诊断标准中,Yamaguchi标准具有较高的敏感性和特异性.皮肤受累为成人Still病的主要症状之一,典型的一过性皮疹为Yamaguchi标准的一项重要表现.非典型皮疹具有多形性,包括持久性丘疹,斑块、荨麻疹样、皮肌炎样皮疹等.持久性丘疹,斑块及荨麻疹样皮疹特征性的临床及病理表现有利于疾病早期诊断,而持久性丘疹,斑块与皮肌炎样皮疹可能与疾病严重程度、预后相关.     

伴持久性色素斑块的成人Still病一例

56岁男性患者,反复高热,胸腹部持久性色素斑块伴瘙痒7个月.皮肤科情况:胸腹部见大致对称分布的色素沉着性苔藓样斑块.皮损组织病理示:表皮角化过度伴角化不全,棘层轻度肥厚,棘层上部可见散在角化不良细胞,真皮浅层可见嗜色素细胞及色素颗粒.诊断:伴持久性色素斑块的成人Still病.     

表现为鞭笞样红斑的成人Still病一例

成人Still病(Adult-onset Still Disease,AOSD)是一种少见的急性系统性炎症性疾病,以间断性高热、粒细胞增多、关节痛或关节炎及皮疹为特征。皮肤损害是早期确诊AOSD的重要线索,典型的皮损表现为与热峰同时出现和消退的一过性丘疹或斑疹,不伴瘙痒。除此之外,AOSD皮肤受累还可以表现为持久性瘙痒性斑块等非典型皮损,呈荨麻疹样、鞭笞样等[1],可以单独或与典型皮损同时出现[2]。表现为鞭笞样红斑的AOSD临床上少见,现将我科诊治的1例报道如下。     

肉芽肿性口周皮炎二例

报告2例肉芽肿性口周皮炎.例1男,6岁.面部红斑、丘疹5个月就诊.曾外用糖皮质激素软膏后皮疹渐增多.例2女,28岁.口周、鼻周红斑、小丘疹2个月余就诊.2例均表现为面部腔口周围直径1 ～3mm大小的淡红色坚实小丘疹,皮损组织病理检查:真皮毛囊周围上皮样细胞肉芽肿改变.结合临床表现及组织病理学检查,2例均确诊为肉芽肿性口周皮炎.例1口服琥乙红霉素颗粒200 mg/次,每日4次、1％克林霉素凝胶和0.03％他克莫司软膏外用,治疗2个月后皮疹完全消退,1个月后口周皮疹又复发.例2在行组织病理活检后皮疹自行消退,提示本病具有临床自愈倾向.     

成人Still病的研究进展

成人Still病是一种炎症性自身免疫性疾病,其特征是高热、一过性皮疹、关节炎、肝脾肿大、多系统累及和实验室异常,包括中性粒细胞增多和高铁蛋白血症.研究表明,成人Still病患者促炎症细胞因子IL-1β、IL-6、IL-18均有升高,这些细胞因子诱导与维持Th17功能相关联.成人Still病的治疗主要包括非甾体抗炎药、糖皮质激素和抗风湿药物.生物制剂也常用于治疗成人Still病,不仅在治疗方面有应用前景,尚可部分解释该病的发病机制.     

进行性骨发育异常三例分析

目的 探讨进行性骨发育异常的临床病理特征、诊断及治疗.方法 分析3例进行性骨发育异常患儿的临床病理资料,并复习相关文献.结果 3例患儿均为女性,出生后数天至数月发病,临床表现为头皮、躯干、四肢质硬的淡红色或肤色丘疹、结节或斑块,无明显症状.组织病理学检查均显示真皮中深层成片的骨或骨样组织形成.3例患儿血清钙、磷及甲状旁腺激素水平均未见明显异常.例1口服碳酸氢钠片并切除右下腹皮疹,随访约7个月右下腹皮疹无复发,但其他皮疹较前凸出、增大、变硬,并出现新发皮疹;例2外用阿达帕林凝胶,皮疹未增多,但皮损增大,质地变硬;例3未治疗,已失访.结论 进行性骨发育异常临床表现为向深部进行性发展的质硬的斑丘疹、斑块,目前尚无有效方案预防或治疗.     

疣状表皮发育不良并发鲍恩样丘疹病

报告1例疣状表皮发育不良并发鲍恩样丘疹病.患者女,39岁.面部、四肢出现褐色斑片26年,大、小阴唇及肛周出现深竭色丘疹、斑块2年.面部皮损组织病理检查示表皮呈网篮状角化过度,表皮上部可见凹空细胞,其细胞核固缩,符合疣状表皮发育不良组织病理改变.会阴部皮损经组织病理检查确诊为鲍恩样丘疹病.     

疣状表皮发育不良并发鳞状细胞癌

报告1例表现为脂溢性角化病样皮损的疣状表皮发育不良并发鳞状细胞癌.患者男,52 岁.四肢皮肤丘疹、斑块20余年,右手背溃疡2 个月.右前臂斑块组织病理检查:同时有疣状表皮发育不良及脂溢性角化病的表现.右手背溃疡组织病理表现符合高分化鳞状细胞癌.     

硬化萎缩性苔癣53例临床及病理分析

目的：探讨硬化萎缩性苔癣（1ichensclerosusetatrophicus，LS）的临床和组织病理特征。方法：分析53例Ls患者的皮疹特点及组织病理学改变情况。结果：本组患者中，男3例，女50例，男：女=1：16．7，平均年龄47．5岁，平均病程2．45年。皮疹特点：早期为群集瓷白色或象牙白色的丘疹或斑块，表面硬化萎缩，毛囊性角化栓，触之较硬，后期皮损出现羊皮纸样萎缩，可伴瘙痒或疼痛感。组织病理显示：外阴以外的4例中均可见毛囊角栓；35例出现不同程度的基底液化变性；41例可见不同程度的毛细血管扩张；真皮内胶原纤维均质化；3例出现基底细胞排列紊乱；细胞核大浓染及不典型增生。结论：皮疹特点和组织学病理改变为Ls临床诊断依据，应加强认识以指导临床诊治。     

伴毛囊黏蛋白沉积的向毛囊性蕈样肉芽肿

报告2例伴有毛囊黏蛋白沉积的向毛囊性蕈样肉芽肿.例1.女,39岁.面部、躯干、四肢出现红斑、丘疹,伴瘙痒3年余就诊.例2.男,52岁.躯干、四肢出现红斑、斑块1年就诊;结合组织病理检查、免疫组化染色及淋巴细胞克隆性基因重排检测.2例患者均诊断为伴有毛囊黏蛋白沉积的向毛囊性蕈样肉芽肿.     

Persistent pruritic papules and plaques associated with adult‐onset Still's disease: Report of six cases

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.     

Adult-onset Still's disease with prurigo pigmentosa-like skin eruption

A 34-year-old woman with adult-onset Still's disease (AOSD) developed prurigo pigmentosa-like lesions on her chest and upper back in addition to the typical rash of AOSD. A biopsy specimen taken from the upper back showed characteristic features of prurigo pigmentosa. The eruption and fever subsided immediately after the administration of 40 mg/day prednisolone, but arthralgia persisted even after intravenous pulse methylprednisolone therapy in combination with immunosuppressive drugs. Various atypical skin rashes, including prurigo pigmentosa-like lesions, have been reported in association with AOSD. Therefore, one should carefully follow the clinical course of a patient in order not to overlook these atypical cutaneous manifestations of AOSD.     

慢性特发性组织细胞增多症一例

患者男,34岁。因双侧腋部皮肤溃疡久不愈合,伴疼痛1年,胸闷、乏力、多尿3年就诊。具有尿崩症、突眼、皮肤黏膜病变（包括浸润结节和溃疡斑块,特别是在腋下,生殖器区域）。其他尚见广泛的皮疹、脱屑或结痂性丘疹。组织学特点为真皮全层弥漫分布的朗格汉斯组织细胞。瘤细胞表达CDla、S-100。组织病理及免疫组化检查符合朗格汉斯组织细胞增生症（Hand—Schuller-Christian病）。本病目前尚无特异性的治疗方法。多系统病变采取化疗,单发病灶者手术及放疗疗效好。     

Clinical and histopathological features of cutaneous manifestations of adult‐onset Still disease

Adult‐onset Still disease (AOSD) is a rare autoinflammatory syndrome characterized by recurring fevers, arthralgia, and consistent laboratory abnormalities that include leukocytosis and hyperferritinemia. Skin findings accompany the disease in nearly 90% of the cases. Early reports described evanescent, pruritic, salmon‐pink or urticarial lesions, referred to as the typical eruption of AOSD. Histopathologic findings consist of superficial perivascular dermatitis with varying number of interstitial neutrophils. Later reports described a more persistent rash that tended to be photodistributed, hyperpigmented, often in a linear configuration, sometimes in a rippled pattern, referred to as the atypical eruption of AOSD. The presence of individual necrotic keratinocytes in the upper spinous layer has been the consistent histopathologic finding. The persistent rash may not represent an atypical presentation of AOSD as recent reports indicate a high prevalence of the rash. Emerging data also suggest that patients with persistent eruption have a worse prognosis. The recognition of the clinical and histopathological findings of skin eruptions of AOSD may facilitate an earlier diagnosis, potentially improving disease outcome. Herein, clinical and histopathological features of cutaneous manifestation of AOSD in 2 Asian women are highlighted accompanied by a relevant review of the disease.     

Persistent plaques and linear pigmentation in adult-onset Still's disease.

A 25-year-old Japanese man presented with high spiking fever, arthralgia and a skin rash. A pruritic edematous erythema with persistent plaques was found mainly on the trunk; these lesions persisted even when the fever subsided, with prominent linear pigmentation. As marked neutrophilia and a high level of serum ferritin were detected, a diagnosis of adult-onset Still's disease (AOSD) was made, even though the persistent eruption was not characteristic of the disease. Oral prednisolone, together with low-dose methotrexate, was given with good results. In the literature, a similar atypical rash has been reported in 11 cases in Japan. All of them required high-dose administration of corticosteroids or other immunosuppressive agents. Severe systemic complications were seen in 3 patients, and 2 cases died of the disease. Persistent plaques and linear pigmentation are some of the manifestations of AOSD, which cannot be overlooked. This appearance could be an indication that suggests an increased risk of systemic complications and a prolonged time to clinical remission.     

Adult‐onset Still disease with peculiar persistent plaques and papules

Adult‐onset Still disease (AOSD) is a systemic inflammatory disorder characterized clinically by high spiking fever, polyarthralgia/arthritis, a salmon‐pink evanescent rash, predominantly neutrophilic leucocytosis, lymphadenopathy, liver dysfunction, and splenomegaly. Recently, a nonclassic, nonevanescent skin rash has been reported. We report a 27‐year‐old woman with AOSD showing persistent pruritic papular lesions. Histologically, dyskeratotic keratinocytes were seen in the upper epidermis. We describe this case in detail and review the previous literature. Nonclassic pruritic eruptions with characteristic dyskeratotic keratinocytes might provide an important clue for the diagnosis of AOSD.     

恙虫病皮肤损害的特征

目的 分析恙虫病皮肤损害的临床特征。方法 分析17例住院恙虫病患者的皮肤损害及临床特征。结果 恙虫病有88.2%（15/17）的患者出现皮肤焦痂及溃疡,有64.7%（11/17）的患者伴有皮肤出疹。焦痂出现在发热后4 ～ 10 d,其发生部位不一,数量为1个,平均直径1.2 cm（0.5 ～ 3.0 cm）,表面干燥呈黑色,周边绕以环形红晕,不伴疼痛及瘙痒,于出现后5 ～ 9 d焦痂脱落形成浅溃疡。出现焦痂者中有80.0%的患者伴有焦痂附近浅表淋巴结肿大。66.7%的患者首诊时未发现焦痂。伴有皮肤出疹者为充血性丘疹（45.5%）和斑丘疹（54.5%）,不伴瘙痒,于出疹后2 ～ 5 d消退。结论 恙虫病的皮肤损害包括皮肤焦痂和皮疹。皮肤焦痂是本病的重要特征,为临床诊断的主要依据。     

Histopathology of Gottron's papules--utility in diagnosing dermatomyositis

Dermatomyositis (DM) is an uncommon connective tissue disease that presents with a characteristic violaceous skin eruption as well as proximal muscle weakness, primarily of the upper extremities. Cutaneous stigmata of DM include Gottron's papules, similarly colored papules and plaques overlying the extensor surfaces of finger joints. While biopsy of the typical poikilodermatous skin eruption found in patients with suspected DM is a standard algorithmic component in the workup and diagnosis of the disease, Gottron's papules are rarely sampled for histopathologic assessment. The precise reason for this is not known but may be related to problems associated with healing because of constant motion forces in the vicinity of the joint. Given this, sparse literature is available on the histopathologic features of Gottron's papules. In this study, we present two cases in which the presence of papular (Gottron's papules) lesions on the fingers led to a presumptive diagnosis of DM and prompted biopsies of the same. The study illustrates the diagnostic utility of biopsies from Gottron's papules.