Generalized Pustular Psoriasis (GPP)Symptoms, Doctors, Treatments, Advances & More
Learn About Generalized Pustular Psoriasis (GPP)
Generalized pustular psoriasis (GPP) is a severe form of a skin disorder called psoriasis. GPP and other forms of psoriasis are caused by abnormal inflammation. Inflammation is a normal immune system response to injury and foreign invaders (such as bacteria). However, when inflammation is abnormal and uncontrolled, it can damage the body's tissues and organs. Individuals with GPP have repeated episodes in which large areas of skin become red and inflamed and develop small pus-filled blisters (pustules). The skin problems can be accompanied by fever, extreme tiredness (fatigue), muscle weakness, an increased number of white blood cells, and other signs of inflammation throughout the body (systemic inflammation). The inflammation problems subside and reappear often. Episodes can be triggered by infection, exposure to or withdrawal from certain medications, menstruation, or pregnancy, although the trigger is often unknown. GPP can be life-threatening if not treated.
Mutations in several genes, including IL36RN and CARD14, increase the risk of developing GPP. These two genes provide instructions for making proteins that play roles in regulating inflammation, particularly in the skin. The IL-36Ra protein, produced from the IL36RN gene, blocks the activity of specific proteins that trigger signaling pathways to promote skin inflammation. IL36RN gene mutations involved in GPP reduce the amount of IL-36Ra protein in the skin. Without control by IL-36Ra, signaling pathways that promote inflammation are overly active.
GPP is the rarest form of psoriasis. Although the worldwide prevalence of GPP is unknown, the condition is estimated to affect 2 per million people in Europe. It also occurs in approximately 0.6 per million people each year in Japan.
When associated with IL36RN gene mutations, risk of GPP is typically inherited in an autosomal recessive pattern, which means both copies of the gene in each cell have mutations. The parents of an individual with an autosomal recessive condition each carry one copy of the mutated gene.
Central Connecticut Dermatology PLLC
Bruce Strober is a Dermatologist practicing medicine in Cromwell, Connecticut. Dr. Strober is rated as an Elite provider by MediFind in the treatment of Generalized Pustular Psoriasis (GPP). He is also highly rated in 13 other conditions, according to our data. His clinical expertise encompasses Psoriasis, Plaque Psoriasis, Pustular Psoriasis, and Generalized Pustular Psoriasis (GPP).
Taubman Center
Johann Gudjonsson, MD, PhD is the Arthur C. Curtis Professor of Molecular Skin Immunology and the Frances and Kenneth Eisenberg Emerging Scholar of the Taubman Medical Research Institute. A graduate of the University of Iceland Medical School, he completed his internship and dermatology residency training at the University of Michigan. Dr. Gudjonsson has served on the faculty of the University of Michigan Department of Dermatology since 2008. He provides general medical dermatology patient care at the University of Michigan Taubman Center and maintains clinical and research interests in inflammatory and autoimmune disorders involving the skin.Dr. Gudjonsson came to the U-M in 2003 as part of an innovative research residency training program. Building upon investigative dermatology training completed in Iceland, Dr. Gudjonsson has been performing basic immunological and genetic work on psoriasis at the U-M. Dr. Gudjonsson's primary research focus is basic immunological and genetic research on psoriasis, with projects directed at improving the diagnosis and treatment of psoriasis. He received the 2007 Young Investigator Award from the American Academy of Dermatology, was named the 2018 Rising Star Lecturer for the Society for Investigative Dermatology and was recipient of the American Skin Association Research Achievement Award in Psoriasis in 2021. Dr. Gudjonsson's research has been supported by research awards from the National Institutes of Health (NIH), the American Skin Association, Dermatology Foundation, Doris Duke Foundation, among others. Dr. Gudjonsson is rated as an Elite provider by MediFind in the treatment of Generalized Pustular Psoriasis (GPP). He is also highly rated in 40 other conditions, according to our data. His clinical expertise encompasses Benign Chronic Pemphigus, Psoriasis, Darier Disease, Cutaneous Lupus Erythematosus (CLE), and Vitrectomy. Dr. Gudjonsson is board certified in Dermatology.
Icahn School Of Medicine At Mount Sinai
Mark Lebwohl is an Internal Medicine provider practicing medicine in New York, New York. Dr. Lebwohl is rated as an Elite provider by MediFind in the treatment of Generalized Pustular Psoriasis (GPP). He is also highly rated in 34 other conditions, according to our data. His clinical expertise encompasses Plaque Psoriasis, Psoriasis, Pustular Psoriasis, and Generalized Pustular Psoriasis (GPP).
Summary: This is a multicenter, randomized, double-blind, placebo-controlled phase II clinical study, all subjects need to use TQH2929 injection/placebo. The aim was to demonstrate the efficacy and safety of TQH2929 injection in patients with acute exacerbations of generalized pustular psoriasis, with a total of 36 subjects.
Summary: Prospective, observational registry for subjects with GPP under the care of a dermatology investigator.Approximately 200 subjects and 75 clinical sites in North America will be recruited to participate with no defined upper limit for either target
Published Date: May 01, 2017
Published By: National Institutes of Health