家族性良性天疱疮1例及家系调查

1 病历摘要 患者男,56岁.腹股沟反复潮湿糜烂、疣状增生20余年,于2006年8月4日就诊于皖南医学院第二附属医院皮肤性病科.20多年前患者双侧腹股沟出现潮湿、糜烂伴瘙痒,多次就诊于他院,分别以"慢性湿疹、股癣及尖锐湿疣"等诊治,症状有所减轻但从未痊愈,且反复加重,累及腹股沟及肛周,并逐渐出现疣状增生.     

青鹏软膏治疗乏脂性湿疹患者的临床对照研究及对皮肤屏障功能的影响北大核心CSCD

乏脂性湿疹又称冬季瘙痒症,是皮肤科常见疾病,主要表现为皮肤缺少弹性、失去光泽伴脱屑、瘙痒等,严重影响患者生活质量。临床常采用糖皮质激素类药物治疗,但长期使用易产生不良反应。青鹏软膏是近年来应用于治疗皮炎湿疹的中药制剂,具有活血化瘀、消炎止痒的功效,可有效控制患者症状,促进皮肤屏障功能恢复。本研究探究青鹏软膏治疗乏脂性湿疹患者的疗效及对皮肤屏障功能的作用。     

血管淋巴样组织增生伴嗜酸性粒细胞增多1例

1 临床资料 患者女,24岁,因"右侧面、耳部皮肤结节1年"来我科就诊.1年前患者右侧耳廓皮肤出现数个米粒大小丘疹,呈淡红色,自觉瘙痒.结节渐增大,增多并且波及周围皮肤及外耳道.曾在当地医院就诊,考虑:"湿疹"抗过敏治疗,无明显效果.因瘙痒明显,影响睡眠来我科进一步诊疗,系统检查未见明显异常.     

湿疹皮炎中西医结合诊疗共识（2009年讨论稿）

湿疹、皮炎是由多种内部或外部因素引起的一组炎症性皮肤病.湿疹为一定义含糊的用词,泛指一些病因不明的急性、慢性皮肤炎症,包括皮肤出现红斑、丘疱疹、渗出、结痂等急性皮炎状态,继之出现丘疹、脱屑、苔藓化、色素沉着或色素减退等慢性过程.对于大部分病因明确的湿疹、皮炎,则常常命名为某某皮炎,如接触性皮炎、日光性皮炎,一些病因不清楚或病因比较复杂时,仍沿用湿疹.     

胫前大疱性表皮松解症一例

患者男,19岁.反复双小腿胫前皮疹、水疱伴瘙痒4年余.患者15岁开始无明显诱因出现双小腿胫前皮肤丘疹及大小不等水疱,伴较剧烈瘙痒,搔抓或碰撞、摩擦后皮损加重,且自觉夏季症状较重,冬季自行减轻.在当地医院曾按"湿疹"处理,疗效不佳,症状仍反复发作.患者否认家族中有类似患者.     

色素异常性皮肤淀粉样变性1例

患者女,21岁.因颈部、躯干、四肢密集丘疹伴色素减退斑13年就诊.患者13年前患者无明显诱因颈部出现密集红色丘疹,消退后遗留点状白斑,随后躯干、四肢等处陆续出现点状色素减退斑,同时全身皮肤变黑、变厚,偶有轻度瘙痒.2000年于某医院行皮肤病理学检查诊断为"色素减退性白斑",未系统治疗.9年前日晒部位如前臂、小腿、颈部出现红斑、丘疹、水疱,轻度瘙痒,以"日光性皮炎"、"湿疹"治疗可治愈,皮疹逐年复发、加重.否认其他疾病病史,无药物过敏史及家族遗传病史.     

家族性良性慢性天疱疮1例误诊分析

正1临床资料患者,52岁,女,因"外阴皮肤红斑糜烂伴瘙痒、疼痛10年"入院。患者自述约10年前开始不明诱因双侧外阴皮肤出现红斑,伴有明显瘙痒,初发病时皮损面积小,瘙痒能忍,未作任何治疗。病情渐进性加重后,即反复在当地医院就诊,考虑为湿疹,但疗效差。病来无尿频、尿急、尿痛等症状。曾就诊于我院妇科,行分泌物涂片检查未见杆菌及球菌等感染。既往史:既往体健,家庭成员中无类似病史。查体:一般情况好,心肺腹无明显异常。双大腿     

氦氖激光联合药物治疗创伤后湿疹的疗效及安全性研究北大核心CSCD

创伤后湿疹（post-traumatic eczema）是因创伤后创面局部皮肤出现炎症反应,引起创伤局部及附近皮肤瘙痒、红斑、丘疹、水疱及糜烂渗出等湿疹样病变。湿疹的治疗主要以外用糖皮质激素为主,但治疗不当会使创伤后湿疹的病程延长,迁延难愈,治疗更加困难。近年来,我科针对创伤后湿疹采用氦氖激光联合常规药物治疗,在治疗中取得满意效果,且方法简便,安全。荆门市第一人民医院皮肤性病科于2013年6月至2014年12月,对住院部治疗的创伤后湿疹患者进行临床观察,现报道如下.     

表现为肉芽肿性酒渣鼻的淋巴瘤肉样瘤反应

淋巴瘤患者有50%伴有皮肤表现.特征性皮疹为恶性细胞皮肤浸润,而非特征性皮疹则不含有恶性细胞浸润,可在淋巴瘤过程中暂时性出现.全身性瘙痒可能是淋巴瘤最常见的非特异性皮肤症状,其它表现有瘙痒样丘疹、多形性红斑、大疱性类天疱疮,结节性红斑、剥脱性皮炎、后天性鱼鳞病、湿疹、色素沉着、苍白色、荨麻疹和紫癜等.近来有人报告淋巴瘤表现为皮肤肉芽肿和肉样瘤样反应.本文报     

系统性间变性大细胞淋巴瘤伴获得性鱼鳞病1例

1 临床资料 患者男，68岁。 于1998年起全身皮肤反复出现湿疹，2005年4-月头面、躯干及叫肢突发大片浸涧性红跚及多处糜烂，找科以“泛发性湿疹，帮样肉芽肿？”收住院，当时的系统检查未见明显异常，红斑浸润处活检表现为湿疹的组织病理像经糖皮质激素、雷公藤多忤片、中药清热除湿等联合治疔3周皮疹消退，     

Neurilemmomatosis--a sporadic and familial cases

Three cases of neurilemmomatosis are reported. A 22-year-old man without any relatives with similar symptoms visited our clinic, complaining of multiple skin tumors since the age of 15 and bilateral acoustic nerve symptoms since 19. Physical examination revealed no pigmented or depigmented spots. Histopathological examination of the eight tumors excised from the skin, acoustic nerve and spinal cord showed that these were all neurilemmomas. A 36-year-old man with a 15 year history of multiple skin tumors and one year history of acoustic nerve symptoms was seen at our clinic, revealing no pigmentary disorders. The tumors excised from the skin and bilateral acoustic nerves were all neurilemmomas histopathologically. A 5-year-old boy, who was the only child of the second case and had had several skin tumors since his birth, visited us after postoperative death of his father. He revealed no pigmentary abnormalities. The histology of the skin tumor was neurilemmoma. The absence of neurofibromas and pigmented spots in these patients with neurilemmomatosis suggests that this disorder might be close to, but distinct from neurofibromatosis. Although familial cases of neurilemmomatosis like our case 2 and 3 reported so far are very few, they support a possibility that neurilemmomatosis might be a genetically determined neurocutaneous syndrome, a kind of phacomatosis.     

Early detection of alkaptonuria

Alkaptonuria is a rare disorder of metabolism characterized by deficiency of homogentisic acid oxidase. This leads to the characteristic features like darkening of urine, ochronosis and arthropathy. Darkening of urine is one of the first symptoms noticed by the parents of the child suffering from this disorder. Ochronosis is seen in various organs like eyes, skin, tendons and joints. A case of 10 year old boy is reported who was brought to this clinic with the presenting complaint of bluish discoloration of sclerae. This discoloration led to eliciting positive history of dark urine off and on. Further investigations confirmed alkaptonuria.     

Changes in neurosyphilis presentation: a survey on 286 patients

Although neurosyphilis (NS) keeps plaguing worldwide, often with oligosymptomatic and atypical manifestations, the most recent reports fail to provide useful information, like details of the clinical history and even of the previous early therapy. We conducted a survey of the literature of the last 5 years on the clinical presentation of NS, recording the aforementioned inaccuracies. One hundred and thirty‐seven articles were collected, reporting on 286 patients. General paresis was the commonest form (49%), often manifesting with cognitive impairment and psychiatric symptoms. Syphilitic meningitis was found in 63 patients (22%), mainly with ocular or auditory involvement. Meningovascular and tabetic form were both found in 12% of cases. Gummatous and epileptic manifestations were rare. Perusal of the literature confirms that NS prevalence is increasing, often with manifestations that are atypical for timing and type of lesions. Unfortunately, many articles are lacking of critical information, like an accurate clinical history and timing of the therapy making difficult to assess the effectiveness of penicillin in preventing NS.     

Atopic dermatitis, nickel sensitivity and xerosis as risk factors for hand eczema in women

Knowledge of the relationship between a history of atopic dermatitis and nickel sensitivity as risk factors for hand eczema is limited. Our objective was to study the relative importance of atopic manifestations and nickel sensitivity as risk factors for hand eczema in women. From women referred for patch testing with a dental series. 100 consecutive nickel-positive and 95 nickel-negative patients were studied. A history of atopic manifestations and a history of 5 symptoms of hand eczema, i.e., dry erythemas, maculopapules, vesicles, erosions and hyperkeratoses were recorded. A history of atopic dermatitis was found lo increase the risk of the 5 symptoms of hand eczema and sick leave due to hand eczema highly significantly. Nickel sensitivity increased only the risk of vesicles and erosions, Xerosis increased the risk of dry erythemas and vesicles. We concluded that a history of atopic dermatitis was more important than nickel sensitivity as a risk factor for hand eczema in women.     

Keratotic vascular papules over the feet: a case of Waldenström’s macroglobulinaemia‐associated cutaneous macroglobulinosis

Waldenström’s macroglobulinaemia (WM) is a plasma‐cell dyscrasia characterized by the monoclonal proliferation of lymphoplasmacytes. A 48‐year‐old man presented with a 4‐year history of multiple painful, hyperkeratotic deep‐seated papules over the pressure areas of both soles. He had a 1‐year history of Raynaud’s phenomenon, intermittent epistaxis, recurrent vomiting, tingling and numbness, and visual disturbances. Histological examination of a skin biopsy found amyloid‐like deposits in the upper and mid dermis involving dermal blood vessels, but apart from periodic‐acid–Schiff, various stains gave negative results for amyloid. Direct immunofluorescence was positive for IgM antibody. Hence, a diagnosis of WM with cutaneous macroglobulinosis was made. Immunoelectrophoresis found monoclonal IgM kappa antibody, and bone‐marrow examination revealed a lymphoplasmacytoid malignancy. The patient’s systemic systems were attributed to hyperviscosity syndrome associated with WM and the cutaneous papules were identified as deposits of excess IgM antibodies. The patient received five cycles of chemotherapy, resulting in nearly complete resolution of the skin lesions and systemic symptoms.     

PHOTOSENSITIVITY: FAMILIAL INCIDENCE, CLINICAL FEATUEES AND INCIDENCE OF EEYTHROPOIETIC PEOTOPORPHYEIA

SUMMARY. Of 54 patients who had presented with skin sensitivity to sunlight, only 1 was found to have erythropoietic protoporphyria. In the remainder no cause could be found. About half of the patients with non-porphyric photo-sensitivity complained of burning, swelling and itching of the skin and about half were not protected from, sunlight by window glass. All of these clinical features are characteristic of erythropoietic protoporphyria and this underlines the fact that a definite diagnosis of erythropoietic protoporphyria depends primarily on the measurement of porphyrins in the blood.
Among the 54 patients were 47 who had at least 3 episodes of photosensitivity. These 47 patients could be subdivided into 2 groups (A) in which the onset was late (mean age 45 years) and the duration of photosensitivity was less than 35% of the lifespan, and (B) in which the onset was early (mean age 12 years) and in which the duration of symptoms was greater than 35% of the lifespan. Only 3 of the 30 patients in Group (A) had a definite family history of photosensitivity, while 8 of the 17 patients in Group (B) had such a family history ( P >0·05).
This suggests the existence of at least 2 distinct populations within non-porphyric photosensitive patients, namely those of early onset with a genetic aetiology like erythropoietic protoporphyria, and those in whom the disease was acquired in late adult life.     

Chronic active Epstein–Barr virus infection with cutaneous and sinus lymphoproliferation in a white female patient with 25 years' follow‐up: an original case report

Chronic active Epstein–Barr virus infection (CAEBV) is characterized by chronic infectious mononucleosis‐like symptoms associated with very high viral load, as assessed by quantitative polymerase chain reaction. We present an unusual case in a French woman who was followed up over 25 years with cutaneous and sinus lymphoproliferation. This white woman presented with a long history of recurrent cutaneous necrotic papules of the skin, which started during childhood and healed spontaneously with depressed scars. The lesions spread to the left maxillary sinus and were associated with hepatomegaly and splenomegaly with no other visceral locations. Pathological examination of the skin and sinus revealed a dermal monoclonal T‐cell lymphoproliferative disorder, CD7⁺ and CD20^?, with no epidermotropism. T‐cell receptor rearrangement was positive, showing the monoclonality from the first biopsy. This T‐cell proliferation was positive for EBV‐encoded small RNA and was associated with a high EBV viral load. Since then, the patient has been in good health, despite a permanently high EBV viral load. Hydroa vacciniforme (HV)‐like lymphoma and natural killer/T‐cell lymphoma were discussed, but none really fit our case. Natural killer cell lymphoma was ruled out because of the indolent course, but sinus lesions do not exist in HV‐like lymphoma. A therapeutic approach is difficult because of the coexistence of viral infection and monoclonal T‐cell proliferation. Chemotherapy is not efficient and induces immunosuppression, which may worsen the prognosis. Although rituximab may have an immunomodulatory function, it was not effective in our case.     

Atopy and other risk factors for UK dentists reporting an adverse reaction to latex gloves

A study was conducted to assess the significance of a personal history of atopy and other risk factors for UK dentists reporting an adverse reaction to natural rubber latex (NRL) gloves. 2535 dentists completed a self-administered questionnaire and, of these, 1034 (group 1) reported an adverse reaction to NRL gloves and 1501 (group 2) did not. Risk factors investigated were: sex, years in clinical practice, exposure to gloves and a history of atopy or food allergy. The signs and symptoms reported by group 1 dentists were recorded. Logistic regression analysis was used to identify a set of risk factors that produced the most discrete model for a dentist reporting an adverse reaction to NRL gloves. A personal history of atopy was a significant risk factor. Dentists with a history of eczema and hand eczema in childhood were most likely to report an adverse reaction to NRL gloves.     

Photosensitivity in lupus erythematosus, UV photoprovocation results compared with history of photosensitivity and clinical findings

Photosensitivity, one of the presenting symptoms in lupus erythematosus (LE), is still poorly defined and varying prevalence figures have been reported. The possibility of a coexisting photodermatosis, especially polymorphous light eruption (PLE), has often not been taken into account. We report the results of ultraviolet A (UVA) and B (UVB) photoprovocation tests in 67 clinically photosensitive patients who had confirmed discoid LE (DLE), systemic LE (SLE) or subacute cutaneous LE (SCLE). The results are compared with a detailed history of photosensitivity and with clinical and serological findings. A pathological photoprovocation reaction, graded as weak, moderate or strong, was induced with either UVA or UVB in 69% of patients with LE, in 100% of those with SCLE, in 70% of those with SLE and in 64% of those with DLE, but in none of 14 controls. Only 16% of the pathological reactions were strong and long-lasting, resembling LE lesions, while 48% were moderate or weak and transient, clinically like PLE. Fifty-three per cent of the provocation reactions which were biopsied showed a PLE-like histology or a non-specific inflammatory reaction, and most of them were clinically moderate or weak reactions of short duration. In the remaining, mostly clinically strong or long-lasting reactions, the histology was consistent with LE. A history of sunlight sensitivity did not predict a pathological photoprovocation result but a positive association between the presence of SSA/Ro or SSB/La antibodies and a pathological photoprovocation reaction was found. We have shown that PLE coexists with LE and that both PLE- and LE-like lesions can be induced with UV radiation in LE patients.     

Anogenital allergic contact dermatitis, the role of spices and flavour allergy

Background: In patients with vulval or anogenital dermatitis, irritant contact dermatitis is more common than allergic contact dermatitis. The reported frequency and relevance of contact sensitivity in anogenital dermatitis varies greatly. Objective: To determine the frequency and relevance of contact sensitization in a Dutch group of female patients with chronic anogenital complaints. Methods: We reviewed patch test results of 53 women with chronic anogenital complaints, with sole vulval symptoms in 29 women and sole perianal in 5, in whom inflammatory skin diseases like lichen sclerosus, lichen planus, psoriasis, as well as infectious diseases were unlikely or excluded as a cause of their symptoms. All women were tested with the European baseline series plus additional test series according to their personal history. Results: Thirty‐five patients (66%) showed one or more positive test reactions. Seven of these patients (20%) had one or more clinically relevant positive reactions, most often to flavours and spices. Conclusion: A considerable number of patients with anogenital dermatitis have a contact sensitization. Clinically relevant reactions were mainly found to spices and flavours. This is in contrast to the data reported in the literature that shows most contact allergies in vulval patients to ingredients of topical medication.