Disease Profile

Graham-Little-Piccardi-Lassueur syndrome

Prevalence ?
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

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ICD-10

L66.1

Inheritance

Autosomal dominant ?A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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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

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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.

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X-linked recessive ?Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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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.

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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.

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Not applicable

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Other Names (AKA)

Graham Little-Piccardi-Lassueur syndrome; Graham Little syndrome; Piccardi-Lassueur-Little syndrome

Categories

Congenital and Genetic Diseases; Skin Diseases

Summary

Graham-Little-Piccardi-Lassueur syndrome (GLPLS) is a rare type of scarring hair loss. It is most commonly found in otherwise healthy women between the ages of 30 and 70 years. GLPLS is characterized by three features: progressive patchy scarring hair loss of the scalp (cicatricial alopecia), non-scarring thinning of the hair in the armpits and groin (noncicatricial alopecia), and spiky rough bumps based around hair follicles (follicular lichen planus). Sometimes individuals with GLPLS experience itching around affected areas, which can be severe.[1][2][3] The cause of this condition is not known; however, over the years, researchers have suggested theories that it may develop in relation to genetic factors, viral exposures, hormonal changes, immune system issues, stress, and vitamin deficiencies.[1] Treatment focuses on slowing the progression of hair loss and may include corticosteroids, retinoids (medications related to vitamin A), psoralen plus ultraviolet light A (PUVA), antimalarial medications, and antibiotics.[1][2]

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
Perifollicular hyperkeratosis
0007468
Sparse axillary hair
Limited armpit hair
Little underarm hair

[ more ]

0002215
Sparse pubic hair
Decreased sexual hair
0002225
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
Lichenification
0100725
Pruritus
Itching
Itchy skin
Skin itching

[ more ]

0000989

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

    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

    • DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.

      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.

        References

        1. Ting PT. Graham-Little-Piccardi-Lasseur Syndrome. Medscape. May 10, 2016; https://emedicine.medscape.com/article/1073743.
        2. Dyall-Smith D. Graham Little syndrome. DermNet NZ. 2011; https://www.dermnetnz.org/topics/graham-little-syndrome.
        3. Pai VV, Kikkeri NN, Sori T, Dinesh U. Graham-Little Piccardi Lassueur Syndrome: An Unusual Variant of Follicular Lichen Planus. International Journal of Trichology. 2011; 3(1):28-30. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3129120/.