Hyperkeratosis lenticularis perstans

General Information (adopted from Orphanet):

Synonyms, Signs: HLP
flegel disease
Number of Symptoms 10
OrphanetNr: 409
OMIM Id: 144150
ICD-10:
UMLs: C0263420
MeSH: C538377
MedDRA:
Snomed: 28488007

Prevalence, inheritance and age of onset:

Prevalence: No data available.
Inheritance: Autosomal dominant
Not applicable
[Orphanet]
Age of onset: Adult
[Orphanet]

Disease classification (adopted from Orphanet):

Parent Diseases: Other epidermis disorder
 -Rare skin disease
Other genetic epidermal disease
 -Rare genetic disease

Symptom Information: Sort by abundance 

1
 (HPO:0008069) Neoplasm of the skin Occasional [Orphanet] 84 / 7739
2
 (HPO:0000989) Pruritus Frequent [Orphanet] 111 / 7739
3
 (HPO:0008065) Aplasia/Hypoplasia of the skin Occasional [Orphanet] 81 / 7739
4
 (HPO:0000962) Hyperkeratosis Very frequent [Orphanet] 216 / 7739
5
 (HPO:0200042) Skin ulcer Frequent [Orphanet] 138 / 7739
6
 (HPO:0007570) Hyperkeratosis lenticularis perstans 1 / 7739
7
 (HPO:0000006) Autosomal dominant inheritance 2518 / 7739
8
 (HPO:0030350) Erythematous papule Very frequent [Orphanet] 123 / 7739
9
 (OMIM) Hyperkeratotic pink or reddish-brown scaly 1 to 5 mm papules of lower leg and dorsum of foot 1 / 7739
10
 (HPO:0003581) Adult onset 117 / 7739

Associated genes:

ClinVar (via SNiPA)

Gene symbol Variation Clinical significance Reference

Additional Information:

Description: (OMIM) Hyperkeratosis lenticularis perstans is a rare cutaneous disorder occurring in older persons and manifested by multiple benign pink to reddish-brown keratotic papules that primarily affect the extremities. The disorder was first described by Flegel (1958) (summary by Bean, ...
Clinical Description OMIM Bean (1969) reported 3 members of the same family with HLP. Another member of the family was reported by Bean (1972). All 4 affected members in 3 generations had onset between the fourth and seventh decade of scattered ...