角层下脓疱性皮病合并结缔组织病二例

报道2例角层下脓疱性皮病，皮损均表现为红斑基础上的水疱、脓疱，组织病理学特征为角层下脓疱，直接免疫荧光阴性；脓疱疱液细菌培养阴性。患者1，女，53岁，合并类风湿关节炎。口服阿维A，糖皮质激素和羟氯喹治疗。患者2，女，69岁，合并干燥综合症及自身免疫性肝炎，予糖皮质激素及米诺环素治疗。2例患者均好转。     

复方甘草酸苷联合米诺环素治疗掌跖脓疱病临床疗效观察

目的：观察复方甘草酸苷联合米诺环素治疗掌跖脓疱病的临床疗效。方法：将44例掌跖脓疱病患者按就诊顺序随机分为治疗组和对照组,治疗组应用复方甘草酸苷片加米诺环素胶囊口服治疗,对照组仅口服米诺环素胶囊治疗,两组均外用3%硼酸溶液湿敷及复方氟米松软膏,共用药4周,观察两组疗效。结果：治疗组痊愈率与总有效率均显著高于对照组,平均起效时间缩短一倍。结论：复方甘草酸苷联合米诺环素治疗掌跖脓疱病疗效肯定,起效快,值得临床推广应用。     

角层下脓疱性皮病2例临床分析

报道2例角层下脓疱性皮病,男女各1例,男63岁,女34岁,皮损表现为红斑、丘疹、水疱、脓疱,病程均为2年,无明显伴发疾病.组织病理学特征为角层下脓疱,直接免疫荧光检查及脓疱疱液细菌培养均阴性.2例患者分别给予糖皮质激素和甲砜霉素治疗,疗效好.     

角层下脓疱病3例及文献分析

目的探讨中国人角层下脓疱病的临床特点。方法对收集的3例角层下脓疱病患者的临床表型进行详细报道,并将结果与近年来国内发表的10例中国人角层下脓疱病患者进行综合对比分析,总结角层下脓疱病的临床特点。结果 13例患者中,男3例,女10例,平均发病年龄(34.9±18.8)岁,平均病程5年,多在中年发病,病程慢性;无明显相关的伴发疾病,大部分患者都具有不同程度的瘙痒及疼痛症状,少数患者在病程中伴有发热;皮损表现有泛发性和局限性,但不侵犯头面部与黏膜部位,皮疹表现无显著差异性;组织病理学特征为角层下脓疱,直接免疫荧光检查及脓疱疱液细菌培养阴性;治疗手段有多种,总体预后良好。结论角层下脓疱病多发生于中年女性,易误诊,可能与感染因素有关,皮损多分布于躯干及皱褶部位;具有典型的组织病理学表现,免疫病理检查及脓疱疱液细菌培养阴性;多种治疗手段均可获得缓解,光疗法为目前使用较多的新疗法。     

雷公藤多苷联合米诺环素治疗玫瑰痤疮23例临床观察

目的评价雷公藤多苷联合米诺环素治疗红斑、丘疹脓疱期玫瑰痤疮的临床疗效和安全性。方法入选的45例玫瑰痤疮患者通过随机数字表法随机分为两组,治疗组(23例)予口服雷公藤多苷及米诺环素,对照组(22例)仅予口服米诺环素,疗程均为6周。结果治疗结束时,治疗组有效率82.61%,对照组有效率50.00%,差异有统计学意义(P0.05)。治疗过程中均未出现严重不良反应。结论雷公藤多苷联合米诺环素治疗玫瑰痤疮安全而有效。     

角层下脓疱性皮病

报告1例角层下脓疱性皮病.患者女,43岁.因躯干反复出现红斑、脓疱2年,加重1个月就诊.皮肤科检查:颈部、腋窝、腰背部、双上肢及臀部见大片红斑,红斑上有绿豆大脓疱,腰背部皮损中央愈合呈淡红色斑片,周围见环状排列的脓疱.皮损组织病理检查:角层下脓疱形成,疱内见少量炎性细胞和棘层松解细胞,其下可见少许坏死的角质形成细胞.直接免疫荧光检查阴性.结合临床、组织病理及免疫病理检查结果,诊断为角层下脓疱性皮病.     

未成年人角层下脓疱病及文献病例分析

<正>角层下脓疱病是一种慢性良性复发性脓疱性皮肤病,好发于中年妇女,临床上较少见,未成年人发病则罕见。本病诊断主要依靠临床特点(躯干及褶皱部位细小脓疱)、病理检查提示角层下脓疱、免疫荧光阴性等可明确诊断。国内较早为陈汝庚~([1])于1976年报道的两例儿童角层下脓疱病,近几十年陆续报道的未成年人发病病例不超过20例。现将我科近两年接诊的2例及文献报道的8例~([1-8])未成年人角层下脓疱病做临床资料分析,总结出未成年人角层下脓疱病的特     

嗜酸性脓疱性毛囊炎1例

报道1例嗜酸性脓疱性毛囊炎。患者男,53岁。面部反复出现红斑、丘疹、脓疱5年,加重3个月。皮肤组织病理检查:外毛根鞘细胞间水肿,嗜酸性粒细胞、中性粒细胞浸润,毛囊内见脓疱,内含中性粒细胞、嗜酸性粒细胞,真皮内毛囊及血管周围大量嗜酸性粒细胞,中性粒细胞及单一核细胞浸润。诊断:嗜酸性脓疱性毛囊炎。给予雷公藤多苷、米诺环素等治疗后,皮损基本消退。     

浅表性大疱型坏疽性脓皮病

报告1例浅表性大疱型坏疽性脓皮病。患者女，58岁。四肢红斑、水疱、溃疡伴疼痛9d入院。组织病理表现为表皮内有一大脓疱，脓疱两侧表皮内有较多中性粒细胞侵入，两侧表皮有明显细胞间及细胞内水肿，真皮浅层及中层有弥漫性中性粒细胞浸润，有明显核尘，核碎裂，真皮胶原纤维间水肿，皮下组织有部分区域出血。类似于典型坏疽性脓皮病，但是在真皮中的位置更表浅。给予小剂量糖皮质激素，米诺环素，雷公藤多苷治疗，2个月后痊愈。     

坏死性痤疮合并重症马拉色菌毛囊炎成功治愈1例

患者男,42岁。面颈部、前胸、背部反复出现坏死性丘疹十年余。通过临床及组织学诊断为:坏死性痤疮合并重症马拉色菌毛囊炎。给予氟康唑静滴及伊曲康唑、米诺环素口服,1个月后炎性毛囊性丘疹、脓疱消退,坏死皮损恢复正常皮肤。     

Subcorneal pustular dermatosis: a variant of pustular psoriasis.

A patient with clinical lesions typical of subcorneal pustular dermatosis of Sneddon and Wilkinson also had stigmata of psoriasis, namely psoriatic plaques, pitting of nails, and psoriatic arthritis. Histologic studies of the pustular lesions were consonant with pustular psoriasis. Correlating the phenomena of previously reported cases of subcorneal pustular dermatosis with those of our patient, we conclude that subcorneal pustular dermatosis of Sneddon and Wilkinson is but another variant of psoriasis.     

Subcorneal pustular dermatosis in the pediatric age

Subcorneal pustular dermatosis is a rare pustular eruption which occurs mainly in middle-aged women and rarely during childhood. We report a case of a 15-year-old female with a 4-year history of pustular lesions on the proximal region of the upper limbs with subsequent impairment of the trunk. Physical examination revealed small pustules distributed on the trunk and proximal region of the limbs. Histopathology showed a subcorneal pustule and direct immunofluorescence for IgA, IgM, IgG and fibrinogen was negative, confirming the diagnosis of subcorneal pustular dermatosis. The patient was treated with dapsone with good clinical response after one month. Subcorneal pustular dermatosis is a rare condition and there are only isolated cases reported in the literature in pediatric patients. Thus, we discuss the main clinical aspects and treatment response of this condition during childhood.     

Juvenile subcorneal pustular dermatosis: a case report

Subcorneal pustular dermatosis is a chronic, relapsing, pustular dermatosis that usually develops in elderly women. It is rarely seen in childhood and adolescence. The hallmark of the disease is formation of a subcorneal pustule detected on histopathologic examination. Herein we present a 13-year-old girl diagnosed with subcorneal pustular dermatosis based on the typical clinical and histologic findings. Direct and indirect immunofluorescence and serum protein electrophoresis had negative results. The patient partially recovered after 5 weeks of treatment consisting of oral dapsone and a topical steroid ointment.     

Subcorneal pustular dermatosis with seronegative polyarthritis

A 55-year-old woman presented with acute onset of subcorneal pustular dermatosis and a seronegative polyarthritis. There have been a few reports of subcorneal pustular dermatosis associated with arthritis.     

Subcorneal pustular dermatosis and IgA paraproteinaemia: response to both etretinate and PUVA

A non-insulin dependent male diabetic is reported with subcorneal pustular dermatosis associated with intraepidermal IgA deposits and a benign IgA paraproteinaemia. Treatment with dapsone and etretinate was reasonably effective, but etretinate had to be discontinued due to the development of diffuse idiopathic skeletal hyperostosis. His subcorneal pustular dermatosis subsequently flared and was troublesome for 2 years until he was commenced on PUVA, with excellent response.     

Neutrophilic Dermatoses

Sweet syndrome, pyoderma gangrenosum, and subcorneal pustular dermatosis are neutrophilic dermatoses – conditions that have an inflammatory infiltrate consisting of mature polymorphonuclear leukocytes. The neutrophils are usually located within the dermis in Sweet syndrome and pyoderma gangrenosum; however, in subcorneal pustular dermatosis, they are found in the upper layers of the epidermis. Sweet syndrome, also referred to as acute febrile neutrophilic dermatosis, is characterized by pyrexia, elevated neutrophil count, painful erythematous cutaneous lesions that have an infiltrate of mature neutrophils typically located in the upper dermis, and prompt clinical improvement following the initiation of systemic corticosteroid therapy. Classical, malignancy-associated, and drug-induced variants of Sweet syndrome exist. Pyoderma gangrenosum is characterized by painful, enlarging necrotic ulcers with bluish undermined borders surrounded by advancing zones of erythema; its clinical variants include: ulcerative or classic, pustular, bullous or atypical, vegetative, peristomal, and drug-induced. Subcorneal pustular dermatosis is an uncommon relapsing symmetric pustular eruption that involves flexural and intertriginous areas; it can be idiopathic or associated with cancer, infections, medications, and systemic diseases. Since Sweet syndrome, pyoderma gangrenosum, and subcorneal pustular dermatosis share not only the same inflammatory cell but also similar associated systemic diseases, it is not surprising that the concurrent or sequential development of these neutrophilic dermatoses has been observed in the same individual. Also, it is not unexpected that several of the effective therapeutic interventions – including systemic drugs, topical agents, and other treatment modalities – for the management of these dermatoses are the same. The treatment of choice for Sweet syndrome and idiopathic pyoderma gangrenosum is systemic corticosteroids; however, for subcorneal pustular dermatosis, dapsone is the drug of choice. Yet, tumor necrosis factor-α antagonists are becoming the preferred choice when pyoderma gangrenosum is accompanied by inflammatory bowel disease or rheumatoid arthritis. Potassium iodide and colchicine are alternative first-line therapies for Sweet syndrome and indomethacin (indometacin), clofazimine, cyclosporine (ciclosporin), and dapsone are second-line treatments. Cyclosporine is effective in the acute management of pyoderma gangrenosum; however, when tapering the drug, additional systemic agents are necessary for maintaining the clinical response. In some patients with subcorneal pustular dermatosis, systemic corticosteroids may be effective; yet, systemic retinoids (such as etretinate and acitretin) have effectively been used for treating this neutrophilic dermatosis – either as monotherapy or in combination with dapsone or as a component of phototherapy with psoralen andUVAradiation. Topical agents can have an adjuvant role in themanagement of these neutrophilic dermatoses; however, high-potency topical corticosteroids may successfully treat localized manifestations of Sweet syndrome, pyoderma gangrenosum, and subcorneal pustular dermatosis. Intralesional corticosteroid therapy for patients with Sweet syndrome and pyoderma gangrenosum, hyperbaric oxygen and plasmapheresis for patients with pyoderma grangrenosum, and phototherapy for patients with subcorneal pustular dermatosis are other modalities that have been used effectively for treating individuals with these neutrophilic dermatoses.     

Subcorneal pustular dermatosis and crippling arthritis.

A 25-year-old woman has suffered with subcorneal pustular dermatosis and a rheumatoid-like arthritis for the past 11 years. Both her skin lesions and arthralgia have abated with institution of dapsone administration and have worsened with interruption or reduction of the dose. The coexistence of arthritis and subcorneal pustular dermatosis and the response of both to therapy suggest a probable association.     

角层下脓疱性皮病的免疫病理与免疫电镜的观察(附二例报告)

本文报告2例临床表现为角层下脓疱性皮病的患者免疫病理出现IgA沉积于颗粒层的细胞间,免疫电镜也证实IgA沉积的部位是细胞间,可能是角层下脓疱性皮病的一种特殊的免疫学表现,也可能是一新的独立疾患.本文提供一个线索,IgA沉积于颗粒层对脓疱的发生,起着重要的致病作用,同时讨论了本病的归属问题.     

Subcorneal Pustular Dermatosis Treated with Phototherapy

A 12-year-old boy had a 5-year history of recurrent generalized dermatoses with scales, crusts, and pustules. Histologic findings showed subcorneal blisters filled with polymorphonuclear leukocytes typical of subcorneal pustular dermatosis. Dapsone and prednisolone with a topical fluocinolone acetonide did not produce improvement. The skin lesions cleared completely after 11 exposures of UV-B three times a week. After 8 months of follow-up, there has been no recurrence.     

Chronic subcorneal pustulosis with vasculitis: a variant of generalized pustular psoriasis in black South Africans

A rare, but distinctive chronic eruption in six female black South Africans is reported. The original diagnosis of subcorneal pustular dermatosis of Sneddon and Wilkinson in these patients was refuted by the subsequent histological observation of both spongiform pustules and an underlying vasculitis. This may represent a previously undocumented form of generalized pustular psoriasis.