Photodynamic therapy effective for the treatment of actinic keratosis and basal cell carcinoma in bullous pemphigoid patients

Treating skin cancers and extensive actinic keratosis in patients with bullous pemphigoid (BP) can be challenging. Treatment options pose unique risks in these patients as surgical wounds can have delayed wound healing and photodynamic therapy (PDT) may exacerbate their blistering disease. We report the successful use of PDT to treat actinic keratosis and skin cancers in two patients with BP, both of whom had excellent response to PDT and tolerated treatment without any bullous disease flares. Carefully selected patients with skin cancers and stable, well controlled BP can be safely considered for treatment using PDT.     

大疱性类天疱疮患者疱液中趋化因子受体5和可诱导共刺激分子的检测

目的：检测C?X?C趋化因子受体5（CXCR5）和可诱导共刺激分子（ICOS）在大疱性类天疱疮（BP）患者疱液中的水平,探讨其在BP发病机制中的意义。方法收集15例BP患者疱液为实验组,15例Ⅱ度烧伤患者疱液为对照组,采用酶联免疫吸附测定（ELISA）方法分别检测CXCR5及ICOS在两组疱液中的水平。结果实验组及对照组疱液中CXCR5水平分别为（219±145.31）、（147±23.83）ng/L,差异有统计学意义（t=4.577,P<0.05）,但疱液中ICOS水平差异无统计学意义[（30.18±14.86）比（21.43±5.32）ng/L,t=1.628,P>0.05]。结论 CXCR5的表达可能与BP发病有一定关系,而ICOS与BP发病的关系尚需进一步研究。     

外伤诱发自身免疫性大疱性皮肤病三例

目的 总结皮肤外伤诱发自身免疫性大疱性皮肤病的临床特点、组织病理学表现等,探讨其可能的发病机制.方法 分析3例由皮肤外伤所诱发的天疱疮或类天疱疮患者的临床表现、组织病理特点及治疗,结合国内外相关文献讨论与总结.结果 3例自身免疫性大疱性皮肤病患者,女1例,男2例,年龄分别为62、60、71岁,由外伤或手术诱发,与发病间隔时间分别为5周、5周和3d,分别确诊为大疱性类天疱疮(BP180抗体109 U/ml,BP230抗体阴性)、寻常型天疱疮(Dsg1抗体68.8 U/ml,Dsg3抗体219 U/ml)和落叶型天疱疮(Dsg1抗体143 U/ml,Dsg3抗体阴性).经糖皮质激素系统和(或)外用治疗后皮疹均明显好转.结论 外伤可能是自身免疫性大疱性皮肤病的1个诱发因素.对于外伤后伤口愈合不良或皮肤出现红斑、水疱、糜烂,用外伤或手术难以解释时,都应警惕自身免疫性大疱性皮肤病的可能,及时行组织病理或免疫病理检查.     

Epidermolysis bullosa acquisita mit facettenreichem klinischen Bild und erfolgreicher Mycophenolatmofetiltherapie

A 60-year-old woman with epidermolysis bullosa acquisita (EBA) presented with a highly variable clinical picture. First she developed oral erosions and ulcers covered with shaggy epithelial rests. Later she developed pustular and target lesions as well as tense cutaneous blisters. Clinically the alterations were strongly suggestive of aphthae or a pustular dermatosis. Histological and immunological examinations revealed subepidermal blister formation, neutrophilic infiltration, linear deposition of IgG and C3 at the basement membrane in the direct immunofluorescence and the evidence of circulating IgG-antibodies against basement membrane (monkey esophagus) in the indirect immunofluorescence. Thus our working diagnosis was a pustular or nodular form of bullous pemphigoid, as a linear IgA dermatosis was excluded. Indirect immunofluorescence using NaCl-separated primate skin localized the basement membrane antibodies in the base of the artificial blister, while immunoblotting of dermal extracts disclosed binding of serum antibodies to a 290-kDa protein. Bullous systemic lupus erythematosus was excluded, leading to the definitive diagnosis of EBA. The clinical variability of this disease is well known; the polymorphic picture in our patient with erosions, pustules, target lesions and blisters is unique in the literature. The similarity in histology and immunofluorescence of the clinically quite different lesions is another unexpected event. Also the speedy response to therapy with the immunosuppressive agent mycophenolate mofetil has only been described once before. The patient remains in remission after 6 months. Because of the poor response to therapy in most cases, the significant skin lesions are a source of physical as well as emotional distress. Additional complications include stenosis of trachea or bronchi injure.     

Pemphigoid gestationis: treatment with immunoapheresis

Background: Pemphigoid (herpes) gestationis is a rare blistering disease of pregnancy. Topical and systemic corticosteroids and antihistamines are usually sufficient for treatment. Case report: A patient suffering from severe, therapy‐resistant pemphigoid gestationis was successfully treated with the additional use of six sessions of immunoapheresis. Systemic corticosteroids could be tapered and serum autoantibody levels to bullous pemphigoid antigen 2 (BPAG2 = BP180) significantly dropped during this treatment. Conclusion: Immunoapheresis represents a helpful therapeutic option with few side effects for severe cases of pemphigoid gestationis, unresponsive to conventional treatment.     

Immunoadsorption in Dermatology

Immunoadsorption (IA) has been successfully used in a large variety of autoantibody‐mediated disorders. In dermatology, IA is increasingly applied as adjuvant treatment for severe and/or refractory autoimmune bullous diseases. These disorders are characterized by autoantibodies against structural proteins of the skin and/or mucous membranes and include, among others, pemphigus vulgaris, pemphigus foliaceus, and bullous pemphigoid. Autoimmune blistering diseases are associated with a high mortality (pemphigus) or morbidity (bullous pemphigoid) and in particular in pemphigus diseases, treatment is challenging. The pathogenetic role of autoantibodies in most of the immunobullous diseases has been clearly demonstrated, therefore, removal of these autoantibodies is a rational therapeutic approach. IA has been shown to effectively lower the serum autoantibodies and to lead to rapid clinical responses. Most recently, IA has been successfully applied in patients with severe atopic dermatitis and high total serum IgE levels. Here, the different treatment protocols, clinical efficacy, and adverse events are summarized.     

免疫球蛋白E型自身抗体在自身免疫相关皮肤病中的研究进展

【摘要】 大量研究显示,免疫球蛋白E（IgE）不但参与变态反应的发生发展,还可通过多种机制诱发和加重自身免疫反应。IgE型自身抗体已证实可在多种自身免疫相关皮肤病中出现,可能通过与自身抗原结合影响树突细胞、肥大细胞、嗜碱性粒细胞等多种免疫机制参与相关疾病的发生发展。本文综述IgE在系统性红斑狼疮、大疱性类天疱疮以及慢性特发性荨麻疹等自身免疫相关皮肤病的诱发和加重中所起的作用和可能机制,为临床诊治提供理论基础。