Dermatology Consultation Service

咨询服务人员包括我本人、John DiGiovanna博士和2011年加入分支的执业护士Olanda海瑟薇。如上所述，在皮肤病的评估和管理方面提供临床专业知识是对NIH社区的一项非常有价值的服务，2007年获得了NIH主任奖。除了在会诊中看到患者外，我还是其他分支机构发起的几项其他方案的副研究者，以及三项治疗方案的主要研究者（将单独讨论）。我是助理研究者的当前非皮肤科分支方案包括GVHD的自然史;恶性淋巴瘤/白血病的低强度移植;使用无关供体的免疫耗竭疗法和低强度移植; DOCK 8免疫缺陷的同种异体移植;慢性GVHD的泊马度胺;闭塞性细支气管炎的II期孟鲁司特;肾细胞癌的厄洛替尼和贝伐单抗;自身炎症性疾病的自然史;阿那白滞素用于白塞病;阿那西普用于IL-1拮抗剂缺乏。慢性移植物抗宿主病（GVHD）的合作努力是一个主要的多学科合作与几个NCI和非NCI研究人员研究GVHD的自然史。根据我在这个小组的经验，我已经发表了几篇关于皮肤GVHD的文章和临床手稿，并荣幸地向全国各地的各种小组（哈佛，塔夫茨，美国）发表演讲。（美国皮肤病学会、地区皮肤病学会和世界皮肤病大会）通过繁忙的忙碌咨询服务，合作临床研究非常活跃。在患有几种周期性发热综合征的患者中新发现的pyrin基因突变，以及对所产生的自身炎症性疾病有效的生物疗法的可用性，已经将一组新的患者引入临床。我们现在正在系统地描述PAPA综合征、白塞病和IL-1受体拮抗剂（DIRA）缺乏患者的皮肤表现和评估治疗反应。我目前是两个开放性治疗方案的主要研究者：甲磺酸伊马替尼（08-C-0148）用于治疗皮肤慢性GVHD（仅数据分析），阿那白滞素用于治疗嗜中性脓疱性皮肤病（13-C-0071;招募）。

The Consult Service Staff consists of myself, Dr. John DiGiovanna, and a Nurse Practitioner, Olanda Hathaway, who joined the Branch in 2011. As mentioned above, providing clinical expertise in the assessment and management of the cutaneous disease is a highly valued service to the NIH community which was recognized by the NIH Director's Award in 2007. In addition to seeing patients in Consultation, I am an Associate Investigator on several other protocols initiated by other branches as well as a Principal Investigator on three therapeutic protocols (to be discussed separately). The current non-Dermatology Branch protocols in which I am an Associate Investigator include Natural History of GVHD; Reduced intensity transplants for malignant lymphomas/leukemias; Immune-depleting therapy and reduced intensity transplant using unrelated donors; Allogeneic transplant for DOCK8 Immunodeficiency; Pomalidomide for chronic GVHD; Phase II Montelukast for bronchiolitis obliterans; Erlotinib and bevacizumab for renal cell carcinoma; Natural history of auto-inflammatory diseases; Anakinra for Behcet's disease; Rilonacept for deficiency of the IL-1 antagonist. The chronic graft versus host disease (GVHD) collaborative effort is a major multidisciplinary collaboration with several NCI and non-NCI investigators studying the Natural history of GVHD. Based on my experience with this group, I have published several text chapters and clinical manuscripts on cutaneous GVHD and have been honored to lecture to various groups around the country (Harvard, Tufts, U. of Pennsylvania, the American Academy of Dermatology, regional dermatologic societies, and the World Congress of Dermatology.) Collaborative clinical research is extremely active via the busy consultation service. The newly discovered mutations in pyrin genes in patients with several periodic fever syndromes, and the availability of biologic therapies that have efficacy in the resulting autoinflammatory diseases have introduced a new group of patients to the clinic. We are now systematically characterizing cutaneous manifestations and assessing responses to treatment in patients with PAPA syndrome, Behcets and deficiency of IL-1 receptor antagonist (DIRA). I am currently Principal Investigator on two open therapeutic protocols: imatinib mesylate (08-C-0148) for the treatment of sclerotic chronic GVHD (data analysis only), and anakinra for neutrophilic pustular skin disease (13-C-0071; recruiting).