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Disease Profile
Generalized pustular psoriasis
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.
1-9 / 1 000 000
Age of onset
Adult
ICD-10
L40.1
Inheritance
Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.
Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.
X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.
Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.
Not applicable
Other names (AKA)
Psoriasis 14, pustular; GPP
Categories
Congenital and Genetic Diseases; Skin Diseases
Summary
Generalized pustular psoriasis is a severe inflammatory skin condition that can be life-threatening. Affected people develop episodes of red and tender skin with widespread pustules throughout their body. This is generally accompanied by fever, chills, headache, rapid pulse rate, loss of appetite, nausea and muscle weakness. The condition generally resolves within days or weeks; however, relapses are common.[1] Some cases of generalized pustular psoriasis are caused by changes (
Symptoms
This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.
| Medical Terms | Other Names |
Learn More:
HPO ID
|
|---|---|---|
| 1%-4% of people have these symptoms | ||
| Childhood onset |
Symptoms begin in childhood
|
0011463 |
| Cholangitis |
Bile duct inflammation
|
0030151 |
| Elevated C-reactive |
0011227 | |
| Epidermal acanthosis |
Thickening of upper layer of skin
|
0025092 |
| Furrowed tongue |
Grooved tongue
|
0000221 |
| Geographic tongue | 0025252 | |
| Infantile onset |
Onset in first year of life
Onset in infancy
[ more ] |
0003593 |
| Juvenile onset |
Signs and symptoms begin before 15 years of age
|
0003621 |
| Leukocytosis |
Elevated white blood count
High white blood count
Increased blood leukocyte number
[ more ] |
0001974 |
| Nail dystrophy |
Poor nail formation
|
0008404 |
| Neonatal onset | 0003623 | |
| Neutrophilia |
Increased blood neutrophil counts
|
0011897 |
| Oligoarthritis | 0040313 | |
| Parakeratosis | 0001036 | |
| Polyarticular |
0005764 | |
| Psoriasiform dermatitis | 0003765 | |
| Young adult onset | 0011462 | |
| Percent of people who have these symptoms is not available through HPO | ||
| 0000007 | ||
| Erythema | 0010783 | |
| Fever | 0001945 | |
| Pustule |
Pimple
|
0200039 |
Organizations
Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.
Organizations Supporting this Disease
-
National Psoriasis Foundation (NPF)
6600 SW 92nd Ave., Suite 300
Portland, OR 97223-7195
Toll-free: 1-800-723-9166
Telephone: +1-503-244-7404
Fax: +1-503-245-0626
E-mail: getinfo@psoriasis.org
Website: https://www.psoriasis.org/
Learn more
These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.
Where to Start
- Genetics Home Reference (GHR) contains information on Generalized pustular psoriasis. This website is maintained by the National Library of Medicine.
In-Depth Information
- The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
- Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine.
- Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
- PubMed is a searchable database of medical literature and lists journal articles that discuss Generalized pustular psoriasis. Click on the link to view a sample search on this topic.
References
- Generalised pustular psoriasis. DermNetNZ. September, 2014; https://dermnetnz.org/scaly/pustular-psoriasis.html. Accessed 10/27/2015.
- PSORIASIS 14, PUSTULAR. OMIM. November 2014; https://www.omim.org/entry/614204.