成人Still病八例临床分析

目的 观察成人Still病的特征性皮疹及其典型组织病理改变,为成人Still病的早期诊断及治疗提供帮助.方法 分析8例成人Still病患者的临床资料.结果 病程中,8例患者均有一过性皮疹及持久性丘疹/斑块改变,1/8例患者具有荨麻疹样皮疹,3/8例患者具有皮肌炎样皮疹,1/8例患者具有色素性痒疹样皮疹.8例患者皮损组织病理取材于一过性皮疹和(或)持久性丘疹/斑块,病理结果均表现为棘层上部坏死角质形成细胞,真皮浅层血管周围中性粒细胞、淋巴细胞浸润.结论 成人Still病皮疹改变及组织病理改变具有一定特征,可为疾病的早期诊断提供线索.     

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。     

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.     

成人Still病31例临床分析和诊治体会

成人Still病（adult onset Still＇s disease,AOSD）是一种病因未明,累及全身多系统的疾病,由Wissler于1944年首先报道,称Wissler综合征,1946年Fanconi以变应性亚败血症命名本病,又称Wissler-Fanconi综合征,1973年被正式命名为成人still病并一直沿用至今.该病临床以高热,一过性皮疹,关节疼痛为主要表现,但因其症状复杂多样,缺乏特异的诊断指标,容易漏诊,误诊.为了提高对该病的临床特点的认识,本文对近年来我院收治31例成人Still病患者的临床资料进行了回顾性分析.…… 作者单位：陈黎（福建省立医院皮肤科,福建福州,350001）     

[开放获取] 临床无肌病性皮肌炎诊断标准的演进

【摘要】 目前皮肌炎诊断使用最广泛的标准是Bohan和Peter标准，该标准要求患者除典型皮疹外，还须满足至少2条肌炎表现才能诊断皮肌炎。临床无肌病性皮肌炎（CADM）因无肌炎表现而被排除在外，这使CADM的早期研究十分匮乏，不被临床医生所重视。另外，诊断CADM依赖于对皮疹的认识和皮损组织病理表现，因CADM早期皮疹表现不典型，往往被误诊为玫瑰痤疮、脂溢性皮炎、红斑狼疮等其他疾病，从而影响早期诊断和治疗以及对疾病预后的判断。本文梳理CADM的诊断标准，以利于其早期识别和诊断，也为开展相关临床研究提供依据。     

持久色素斑块皮疹成人Still's病1例

患者女,41岁。反复高热、咽痛、关节痛伴持久色素斑块样皮疹1年。血常规示白细胞增高,B超示脾大。皮肤科检查发现躯干、四肢大致对称分布色素沉着性苔藓样斑块。皮损组织病理学表现具有特征性,表现为表皮上部散在角化不良细胞。在排除了感染性疾病、肿瘤及结缔组织病后,诊断为持久色素斑块皮疹成人Still's病。     

成人Still病31例临床分析

目的探讨成人Still病的临床特点。方法回顾性分析本院1988~2003年间确诊的资料完整的31例成人Still病患者的临床资料。结果发热31例,关节痛28例,以大关节游走性疼痛为主。皮疹27例,为红色斑疹、丘疹或荨麻疹样皮疹,以躯干、四肢多见,多与发热平行。咽痛16例,肌痛12例,淋巴结肿大13例,肝大10例,脾大11例,腹痛2例,肺炎7例。白细胞总数均高于正常,贫血18例,C反应蛋白升高17例,类风湿因子阳性3例。26例患者检测血清铁蛋白,有21例升高。血清谷丙转氨酶升高11例,γ球蛋白增高7例。全部患者多次血培养和骨髓培养均阴性。治疗上首选糖皮质类固醇激素和非甾体类消炎药。酌情加用免疫抑制剂和中药治疗。31例患者均获得缓解,疗程半个月~3个月不等。结论成人Still病临床表现复杂,预后较好,但易复发加重,部分患者可能发展成淋巴瘤或其他结缔组织病。应加强随访。     

脂溢性皮炎样皮肌炎：一种特殊类型的皮肌炎

介绍一种特殊类型的皮肌炎——脂溢性皮炎样皮肌炎,其特点为患者有面部脂溢性皮炎样皮疹,常伴有Gottron丘疹、反向Gottron丘疹、技工手和皮肤溃疡,很少累及肌肉。抗黑素瘤分化相关基因5抗体阳性的脂溢性皮炎样皮肌炎患者更易出现肺间质病变/急进性肺间质病变。通过皮疹联合抗体检测可对脂溢性皮炎样皮肌炎进行早期诊断,早期积...     

具有荨麻疹样皮损的其他疾病

荨麻疹是临床上常见的皮肤病,以反复发作的红斑、风团伴瘙痒为特征,也可出现呼吸道、胃肠道症状。除荨麻疹外,其他一些皮肤病或皮肤综合征也可表现为荨麻疹样皮损,易被误诊为荨麻疹,但抗组胺药物常规治疗无效。本文主要综述了色素性荨麻疹、大疱性类天疱疮、妊娠瘙痒性荨麻疹性丘疹和斑块、荨麻疹性血管炎、嗜酸性肉芽肿性多血管炎、嗜中性荨麻疹性皮病等具有荨麻疹样皮肤改变的皮肤病,为临床诊疗提供思路。     

妊娠期和哺乳期皮肤病的安全用药

妊娠期和哺乳期均会出现皮肤瘙痒和皮疹等改变，有的皮肤病确与妊娠有关，如妊娠瘙痒症（妊娠期肝内胆汁淤积）、妊娠多形疹（妊娠瘙痒性荨麻疹性丘疹或斑块）、妊娠痒疹、妊娠瘙痒性毛囊炎、疱疹样脓疱病、妊娠类天疱疮、妊娠线状IgM病等，但也不能忽视与妊娠无关的一般皮肤病，如疥疮、丘疹性荨麻疹等．     

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.     

Refractory macrophage activation syndrome in the setting of adult‐onset Still disease with hemophagocytic lymphohistiocytosis detected on skin biopsy treated with canakinumab and tacrolimus

A 19‐year‐old Caucasian female with adult‐onset Still disease (AOSD) presented for evaluation of an acute clinical decompensation and atypical annular papules and plaques with purpura on the lower extremities. A punch biopsy demonstrated histiocytes with engulfed degenerated erythrocytes and lymphocytes, consistent with hemophagocytic lymphohistiocytosis (HLH). HLH, clinically referred to as macrophage activation syndrome, is a rare complication of AOSD and is life‐threatening. Relevant clinical, laboratory, and histologic features of this diagnosis are reviewed.     

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’s disease as a cutaneous marker of systemic disease

Adult-onset Still’s disease (AOSD) is a rare, systemic, inflammatory disorder characterized by spiking fevers, an evanescent eruption, arthritis, and multiorgan involvement. The disease has been recently classified as a polygenic autoinflammatory disorder at the “crossroads” of autoinflammatory and autoimmune diseases. The highly characteristic salmon-colored eruption is a cutaneous manifestation of a generalized inflammatory reaction and an important diagnostic criterion. In addition to the evanescent eruption, there are atypical persistent papules and plaques in many patients with AOSD. Emerging data suggest that AOSD with this typical evanescent eruption has a different clinicopathologic presentation and clinical course than AODS with atypical cutaneous manifestations.It appears that there are two subtypes of AOSD with different immunologic profiles, including (1) a systemic disease with high fever, organ involvement, and elevated levels of ferritin, and (2) a chronic disease course with arthritis as the predominant finding. These observations provide novel insight into the disease pathogenesis, suggesting that the underlying mechanisms might differ between these two forms, partially explaining the reported differences in drug response.Recent advances in the understanding of AOSD are summarized with a focus on the spectrum of cutaneous manifestations and its relationship to systemic inflammation.     

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。     

Cutaneous Crohn’s Disease in the Pediatric Population

Abstract: Three healthy children with an anogenital skin eruption, all of whom had undergone extensive medical evaluation and treatment, had undetected intestinal Crohn’s disease. Biopsy of the skin showed noncaseating granulomas consistent with cutaneous Crohn’s disease. Inflammatory bowel disease should be in the differential diagnosis of persistent nontender, erythematous papules, nodules, and plaques in the anogenital region.     

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.