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Disease Profile
Pseudopelade of Brocq
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.
Unknown
Age of onset
Adult
ICD-10
L66.0
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)
Pseudo pelade of Brocq; Brocq pseudopelade; Pseudo-pelade of Brocq
Categories
Skin Diseases
Summary
Pseudopelade of Brocq (PBB) is a slowly progressive, chronic condition characterized by scarring hair loss (cicatricial alopecia). There exists some controversy as to whether PBB is a distinct condition or the common final stage or variant of several different forms of scarring alopecias, such as discoid lupus erythematosus (DLE) or lichen planopilaris (LPP). PBB is classified into two categories depending on whether a scarring alopecia is present (burnt-out or end-stage scarring) or not (idiopathic).[1]
The patches of hair loss present in PBB may be single or multiple. They are usually small, discrete, round or oval, and asymmetrical. The underlying skin is typically smooth, soft, and flesh-colored or white, with little, if any, inflammation.[2] Although the exact cause of PBB has not been identified, it is believed to be an
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
|
---|---|---|
80%-99% of people have these symptoms | ||
Alopecia |
Hair loss
|
0001596 |
Lichenification | 0100725 | |
Sparse scalp hair |
Reduced/lack of hair on scalp
Scalp hair, thinning
Sparse, thin scalp hair
sparse-absent scalp hair
[ more ] |
0002209 |
30%-79% of people have these symptoms | ||
Papule | 0200034 | |
Recurrent skin infections |
Skin infections, recurrent
|
0001581 |
5%-29% of people have these symptoms | ||
Abnormality of the nail | 0001597 | |
Aplasia/Hypoplasia of the eyebrow |
Absence of eyebrow
Lack of eyebrow
Missing eyebrow
[ more ] |
0100840 |
Cheilitis |
Inflammation of the lips
|
0100825 |
Treatment
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
-
American Hair Loss Association
23679 Calabasas Road # 682
Calabasas, CA 91301-1502
E-mail: [email protected]
Website: https://americanhairloss.org -
Cicatricial Alopecia Research Foundation (C.A.R.F.)
1586 Sumneytown Pike
PO Box 1322
Kulpsville, PA 19443
Telephone: +1-267-613-9811
E-mail: [email protected]
Website: https://www.carfintl.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
- The North American Hair Research Society (NAHRS) has developed an informative page on cicatricial alopecia. Click on the link to learn more.
- The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.
In-Depth Information
- Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
- 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.
- 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 Pseudopelade of Brocq. Click on the link to view a sample search on this topic.
References
- Kendall M Egan. Medscape. October 30, 2015; https://emedicine.medscape.com/article/1071359-overview.
- Delwyn Dyall-Smith. Pseudopelade of Brocq. DermNet New Zealand. https://www.dermnetnz.org/topics/pseudopelade-of-brocq/. Accessed 9/15/2016.
- Nilofar Diwan, Sneha Gohil, Pragya A Nair. Primary Idiopathic Pseudopelade of Brocq: Five Case Reports. Int J Trichology. 2014 Jan-Mar; 6(1):27-30. https://www.ncbi.nlm.nih.gov/pubmed/?term=25114452.
- Kendall Egan. Brocq Pseudopelade. Medscape. March 10, 2017; https://emedicine.medscape.com/article/1071359-overview.
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