Pilomatricomas in Turner syndrome
Simon Wood1, Dai Nguyen, Kim Hutton
1Department of Burns and Plastic Surgery, Morriston Hospital, Swansea, UK. siwood@doctors.org.uk
Insights
Pilomatricomas, benign skin tumors, are uncommonly associated with Turner syndrome. This case report suggests a potential predisposition in girls with Turner syndrome, warranting clinical awareness.
Area of Science:
- Dermatology
- Genetics
- Pediatrics
Background:
- Pilomatricomas are benign cutaneous tumors originating from hair matrix cells, typically occurring in children.
- While often isolated, pilomatricomas have been linked to various inherited conditions.
- Turner syndrome is a genetic condition affecting females, characterized by chromosomal abnormalities.
Observation:
- A 10-year-old girl with Turner syndrome presented with two pilomatricomas, one of which was unusually large.
- This observation adds to a previous report of pilomatricoma in two patients with Turner syndrome.
Findings:
- The findings suggest a potential, albeit uncommon, association between pilomatricomas and Turner syndrome.
- This association may stem from the hair-bearing skin abnormalities frequently observed in Turner syndrome.
Implications:
- Clinicians managing pediatric cutaneous masses should consider this association.
- Increased awareness may aid in the early diagnosis and management of pilomatricomas in patients with Turner syndrome.
Abstract:
Pilomatricomas are benign, cutaneous tumors of the hair matrix cells, which present predominantly in childhood. Although usually an isolated finding, they have been described in association with several inherited conditions. We report a 10-year-old girl with Turner syndrome who has two pilomatricomas, including one giant tumor. In combination with a previous report of pilomatricoma in two patients with Turner syndrome, our observation indicates that it is likely a real, although uncommon, association of pilomatricomas with Turner syndrome exists. This apparent predisposition may be etiologically related to the abnormalities of hair-bearing skin observed in Turner syndrome. Doctors involved in the diagnosis and management of cutaneous masses in children should be aware of this association.
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