Orphanet: Graham Little Piccardi Lassueur syndrome

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Graham Little-Piccardi-Lassueur syndrome

Disease definition

A variant of lichen planopilaris characterized by the clinical triad of progressive cicatricial (scarring) alopecia of the scalp, follicular keratotic papules on glabrous skin, and variable alopecia of the axillae and groin.

ORPHA:505

Classification level: Disorder

Synonym(s):
- Graham Little syndrome
- Piccardi-Lassueur-Little syndrome
Prevalence: Unknown
Inheritance: -
Age of onset: Adult
ICD-10: L66.1
ICD-11: EA91.2
OMIM: -
UMLS: -
MeSH: -
GARD: 3195
MedDRA: -

Summary

Epidemiology

It is a very rare disease but the exact prevalence is not known. It mainly affects women during adulthood (30-60 years of age).

Clinical description

Scarring alopecia presents as small confluent patches that are atrophic and cicatricial in the center but erythematous and squamous around the edges. Follicular keratosis presents as pruritic, red-brown, follicular spiny papules on the trunk and extremities. Generally, the three clinical features appear simultaneously but in some cases, scalp alopecia precedes the follicular keratosis.

Etiology

Etiology is unknown.

Expert reviewer(s): Dr Stephanie ARNOLD - Dr Susan COOPER - Last update: May 2011

A summary on this disease is available in Deutsch (2011) Español (2011) Français (2011) Italiano (2011) Nederlands (2011) Português (2011) Greek (2011, pdf)

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