恶性萎缩性丘疹病的研究进展

恶性萎缩性丘疹病是一种以萎缩性丘疹样皮损为特征的疾病,常伴有多系统脏器受累,死因多为胃肠穿孔和频发弥漫性腹膜炎.恶性萎缩性丘疹病发病原因尚不明,可能与免疫功能紊乱、高凝状态、纤维溶解蛋白异常、基因易感性、病毒和细菌感染有关.组织病理:血管腔纤维性增生和纤维蛋白血栓形成引起血管阻塞.免疫组化检查提示,膜攻击复合物、基质细胞衍生因子1/趋化因子12等因子有不同程度的沉积.恶性萎缩性丘疹病至今尚无有效的治疗方法,需要进一步研究.     

恶性萎缩性丘疹病二例

报道2例恶性萎缩性丘疹病患者。2例患者具有恶性萎缩性丘疹病的典型皮损,中心为瓷白色萎缩斑,周围有红晕。例1在发病1年半内胃肠道受累,是经典型恶性萎缩性丘疹病,抗心磷脂抗体阳性,病理可见到楔形坏死和血管中透明血栓形成。例2发病6年,目前无系统受累,抗心磷脂抗体阴性,病理表现为血管周围淋巴细胞浸润,大量黏蛋白沉积。2例患者均采用双嘧达莫和阿司匹林治疗,例1系统受累后使用环磷酰胺。     

恶性萎缩性丘疹病1例

患者女,21岁。因全身皮肤丘疹1年余,且逐渐增多,于2005年2月来我科就诊。1年前患者无明显诱因胸、腹部出现红色丘疹,不痛不痒。皮损可溃破,愈后遗留瓷白色萎缩性瘢痕。皮损逐渐增多,波及躯干和四肢。体格检查:一般情况好,系统检查无明显异常。皮肤科检查:胸腹部、背部、四肢散在红色萎缩性丘疹,中央凹陷,为瓷白色,周围毛细血管扩张(图1)。皮损组织病理检查:表皮部分萎缩,其下方真皮胶原纤维呈楔形增生,真皮内灶性黏蛋白沉积。血管周边见较多炎性细胞浸润,未见明显血管病变(图2)。实验室检查:血、尿常规,肝、肾功能均正常。胃镜检查示慢性浅…     

恶性萎缩性丘疹病二例

报道2例恶性萎缩性丘疹病患者.2例患者具有恶性萎缩性丘疹病的典型皮损,中心为瓷白色萎缩斑,周围有红晕.例1在发病1年半内胃肠道受累,是经典型恶性萎缩性丘疹病,抗心磷脂抗体阳性,病理可见到楔形坏死和血管中透明血栓形成.例2发病6年,目前无系统受累,抗心磷脂抗体阴性,病理表现为血管周围淋巴细胞浸润,大量黏蛋白沉积.2例患者均采用双嘧达莫和阿司匹林治疗,例1系统受累后使用环磷酰胺.     

恶性萎缩性丘疹病一例

报道1例恶性萎缩性丘疹病。患者男,58岁。因全身皮损5年余,急腹症剖腹探查术后11 d入院。躯干、四肢散在分布圆形、椭圆形淡红色丘疹,黄豆大小,周边呈环状隆起,境界清楚,中央破溃、萎缩。皮损组织病理示真皮深部小血管周围淋巴细胞浸润,部分小血管管腔闭塞。临床表现及组织病理改变符合恶性萎缩性丘疹病。该病临床罕见,累及系统,死亡率高。     

伴牙齿异常棘状秃发性毛囊炎一例

患者男,24岁.因头皮脓疱、萎缩性瘢痕伴脱发6年.皮肤科检查:头皮弥漫性秃发伴萎缩性瘢痕.头顶仅残留少数头发,发根处有角化性丘疹、脓疱或黑色痂,前额、颞、枕部近发际处尚有部分头发,枕部头发成小簇,发根处见角化性丘疹.眉弓有红斑、鳞屑及毛囊角化性丘疹,眉毛稀疏、脱落.腋毛也稀疏.两颊、腋、胸、腹、后背、四肢均有明显毛囊角化性丘疹.口腔科检查:上下前牙排列间隙过大,上前牙前倾,(+1)先天性缺失,前牙深覆(牙合),深覆盖.沟纹舌,丝状乳头消失.皮损组织病理:表皮棘层增厚,毛囊周围可见大量浆细胞、中性粒细胞及多核巨细胞浸润.结合临床及组织病理表现,符合棘状秃发性毛囊炎的诊断.     

恶性萎缩性丘疹病1例

患者男 ,3 7岁。躯干、四肢皮肤起萎缩性丘疹 2年 ,反复腹膜炎 5个月 ,治疗无效死亡     

Degos病1例

患者女,24岁。躯干及四肢泛发萎缩性丘疹1年。皮损组织病理示:表皮局限性坏死,真皮浅层胶原纤维素样坏死,真皮深层血管壁增厚,管壁内有嗜中性白细胞、淋巴细胞和组织细胞浸润,管腔内血栓形成。血管周围慢性炎症细胞浸润。诊断为Degos病。     

恶性萎缩性丘疹病一例

患者，女，58岁。躯干四肢散在红斑丘疹4个月余。皮肤科检查：躯干四肢散在红斑丘疹，丘疹中央可见瓷白色萎缩性斑片，边缘隆起伴红晕，伴腹痛。皮损组织病理示：表皮萎缩变薄，真皮浅中层胶原变性，血管周围散在小片状的淋巴细胞及组织细胞浸润。诊断：恶性萎缩性丘疹病。给予阿司匹林、双嘧达莫、雷贝拉唑钠、美沙拉嗪、阿嗪米特口服治疗1个月后腹痛症状缓解，全身皮疹颜色变淡。2个月后患者腹痛加重诊断为肠穿孔，术后无明显改善。4个月后去世。     

恶性萎缩性丘疹病1例报告

恶性萎缩性丘疹病(ｍａｌｉｇａｎｔａｔｒｏｐｈｉｃｐａｐｕｌｏｓｉｓ),亦称Ｄｅｇｏｓ综合征。临床罕见,现报告1例如下。患者女,35岁,躯干、四肢泛发萎缩性丘疹2年,下腹疼痛半年于1998年10月26日就诊。患者2年前躯干、四肢开始无诱因成批出现散在淡红色丘疹,约1～2个月丘疹中央萎缩。皮疹逐渐增多,无明显自觉症状,其间病人常有消化不良及便秘,一直未予重视。近半年来下腹不规则疼痛,无呕吐及便血。3月前腹痛突然加重,对症治疗无效,在当地医院急诊行剖腹探查术,术中见腹腔中量稀薄脓液,肠道充血,回肠散在直径2～6ｍｍ白色斑片,分布于大部…     

Psoriasis Verrucosa Showing Peculiar Histologic Features

A 60-year-old man had a 10-year history of linearly arranged verrucous papules on his left fingers in addition to psoriatic erythematosquamous papules and plaques on his trunk and extremities. The verrucous papules were clinically classified into two types: dome-shaped papules with a keratotic plug and crater-shaped papules with a central depression. Histopathologically, the former showed cup-shaped acanthosis with a parakeratotic column containing Munro's microabscesses, while the latter showed a cup-shaped epidermal invagination with Kogoj's spongiform pustule-like changes. Both types of papules were clinically diagnosed as psoriasis verrucosa, but their histologic features differed from those reported previously.     

The effect of haematopoietic stem cell transplant on papules with 'pebbly' appearance in Hunter's syndrome

BACKGROUND: Hunter's syndrome is associated with several cutaneous findings. For instance, papules with 'pebbly' appearance are a specific marker for the disease. However, it remains uncertain whether they disappear after haematopoietic stem cell transplant (HSCT). OBJECTIVES: To investigate the papules with 'pebbly' appearance before and after HSCT in infants with Hunter's syndrome, and to clarify the effect of HSCT on papules. PATIENTS: We observed five Japanese boys with Hunter's syndrome who had received HSCT at 4-11 years of age. RESULTS: The post-HSCT physical examinations revealed that papules disappeared completely within 35 days after the transplant with progressive reduction of cutaneous tightness in all the patients. Histochemical findings showed that papules contained a large amount of hyaluronic acid in the extracellular materials of the dermis and sulphated acid mucopolysaccharides in dermal fibroblasts before HSCT. CONCLUSIONS: These results suggest that papules with a 'pebbly' appearance fade away through the digestion of a large amount of hyaluronic acid in cutaneous tissues by normal tissue histiocytes or enzymes of donor origin at an early stage after HSCT.     

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.     

Multiple rheumatoid papules characteristic of the early stage of rheumatoid vasculitis.

The rheumatoid papule has recently been described as the skin manifestation of rheumatoid arthritis (RA). The papules represent leukocytoclastic vasculitis and necrobiosis clinicopathologically. We report a 59-year-old Japanese woman with multiple recurrent rheumatoid papules located on the bilateral extensor forearms in correlation with the level of RA activity and as a cutaneous manifestation of rheumatoid vasculitis. These papules appeared prior to other cutaneous manifestations. It was suggested that rheumatoid papules represent the early stage of rheumatoid vasculitis and that they are a reliable early clinical marker of rheumatoid vasculitis.     

皮肌炎患者Gottron皮疹与肺间质病变

目的 探讨皮肌炎（DM）患者中Gottron皮疹与肺间质病变（ILD）的关系。方法 回顾分析83例皮肌炎患者的临床资料,根据有无Gottron皮疹分组,比较两组的临床特点。结果 DM患者Gottron皮疹的总阳性率为59%（49/83例）,ILD的发生率为42.2%（35/83例）,Gottron皮疹阳性组ILD的发生率明显低于Gottron皮疹阴性组,两者差异有统计学意义（24.5%比67.6%,P ＜ 0.01）。35例ILD中Gottron皮疹阴性者的CO弥散率（DLCO）明显低于Gottron皮疹阳性者（60.9 ± 13.5比72.3 ± 12.7,P ＜ 0.05）。Gottron皮疹阳性组与阴性组的肌力（4.0 ± 0.9比3.8 ± 1.2,P ＞ 0.05）、肌肉压痛（63.3%比70.6%,P ＞ 0.05）、肌酶水平（肌酸磷酸激酶1047.9 ± 1402.7比1359.7 ± 1752.4,乳酸脱氢酶397.2 ± 226.5比402.4 ± 197.0,AST 97.6 ± 98.0比120.1 ± 82.3,P均 ＞ 0.05）比较差异无统计学意义。两组的ESR、CRP水平比较差异亦无统计学意义（分别为26.5 ± 16.4比33.3 ± 25.3,11.9 ± 8.3比15.5 ± 9.8,P均 ＞ 0.05）。Gottron皮疹阳性组与阴性组恶性肿瘤的发生率差异无统计学意义（21.4%比6.5%,P ＞ 0.05）。结论 Gottron皮疹阳性的皮肌炎患者伴发肺间质病变的可能性小,且病变程度可能较轻。Gottron皮疹与DM的肌肉病变程度及病情活动无明显关系,Gottron皮疹可能与恶性肿瘤的发生无明显关系。     

Effect of a single overnight topical application of miconazole nitrate paste on acne papules

BACKGROUND: The classical management of acne calls for prolonged oral and/or topical treatments; however, some patients request a rapid effect to make the papules disappear within a few hours or days. OBJECTIVE: To test the effect of a single overnight application of a paste containing 0.25% miconazole nitrate on acne papules, and comparison with the effect of the same but unmedicated paste. METHOD: Narrow-band reflectance spectroscopy was used to assess the changes in E index (erythema) after overnight application of the pastes. In the first study, a total of 117 acne papules were assessed in 15 adolescents. Measurements of the E index were performed at 24-h intervals for 4 days on acne papules and the surrounding normal-looking skin. The last two assessments were performed the mornings preceding and following, respectively, a single application of the medicated paste. The second part of the study consisted of a double-blind, split-face study on 25 adolescents with acne. They applied each of the two formulations on a randomized part of the forehead. A total of 161 acne papules received the medicated paste and 160 received the same but unmedicated paste. Measurements were performed the mornings before and after the overnight treatment. RESULTS: In the first part of the study, no significant daily difference was observed between the three series of pretreatment E indices, on both acne papules and normal skin. The treatment did not affect the E index of normal skin. By contrast, it decreased significantly the E index of the acne papules. The second part of the study also showed that the medicated paste significantly decreased the E index of acne papules. This effect was significantly (P < 0.05) superior to that of the unmedicated paste. CONCLUSION: A single overnight application of miconazole nitrate paste appears to reduce the erythematous aspect of acne papules.     

Rheumatoid papules treated with dapsone

A patient with a long history of seropositive rheumatoid arthritis presented with a progressive papular eruption on the trunk. A skin biopsy showed a neutrophilic and palisaded granulomatous dermatitis compatible with rheumatoid papules. The eruption failed to respond to topical and systemic corticosteroids and the patient was treated with dapsone with complete resolution of the rash. Rheumatoid papules are a rare disorder seen in a variety of collagen vascular diseases. The literature concerning the treatment of rheumatoid papules is scanty. In this patient, dapsone was an effective treatment.     

Use of topical rapamycin in acral pseudolymphomatous angiokeratoma of children (APACHE): A report of two cases and review of the literature

Acral pseudolymphomatous angiokeratoma of children (APACHE) is a rare, benign disease characterized clinically by multiple, asymptomatic, erythematous papules in the acral regions. We report APACHE in a 12-year-old girl with erythematous-violaceous papules on the lateral dorsum of her foot and toes, and a 3-year-old girl with erythematous papules on the plantar aspect of her foot. Topical rapamycin ointment improved the lesions and both patients tolerated the medication well. Topical rapamycin appears to be a potentially efficacious, well-tolerated, non-invasive therapy in APACHE, although further studies are needed.