Localization and age distribution of telangiectases in children and adolescents with hereditary hemorrhagic telangiectasia: A retrospective cohort study.
J Am Acad Dermatol
; 81(4): 950-955, 2019 Oct.
Article
in En
| MEDLINE
| ID: mdl-30819528
ABSTRACT
BACKGROUND:
The location of telangiectases in hereditary hemorrhagic telangiectasia (HHT), as set forth in the consensus diagnostic (Curaçao) criteria, is based primarily on adults.OBJECTIVE:
Document the locations and numbers of telangiectases in a cohort of pediatric patients with HHT.METHODS:
A retrospective chart review using a standardized data collection form for site and number of telangiectases was performed for pediatric patients with HHT (age, 0-18 years) from 2005 to 2016.RESULTS:
Of 90 pediatric patients with HHT, 71% had one or more telangiectases. Of all the telangiectases counted (N = 319), cutaneous telangiectases were more common (73%) than oral telangiectases (27%). The hands were the most frequent site, accounting for 33% of all telangiectases. Adolescents were more likely than children to have cutaneous telangiectases (85% vs 50% [Q = 0.005]). The most frequent sites in children younger than 10 years were the hands excluding the fingers (27%), fingers (25%), and face (23%). Only 23% of subjects (21 of 90) presented with multiple (≥3) telangiectases at locations considered characteristic for the current consensus diagnosis guidelines (lips, oral cavity, and fingers).LIMITATIONS:
Ascertainment bias based on recruitment.CONCLUSIONS:
In this pediatric population, telangiectases at sites not included as "characteristic" by the Curaçao diagnostic criteria were common. The Curaçao criteria in regard to both number and location of telangiectases may be inadequate in the pediatric HHT population.Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Telangiectasia, Hereditary Hemorrhagic
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Face
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Hand
Type of study:
Etiology_studies
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Guideline
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Observational_studies
Limits:
Adolescent
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Child
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Child, preschool
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Female
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Humans
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Infant
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Male
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Newborn
Language:
En
Journal:
J Am Acad Dermatol
Year:
2019
Document type:
Article