A Case of Goltz Syndrome / 대한피부과학회지
Korean Journal of Dermatology
;
: 122-125, 2008.
Article
in Korean
| WPRIM
| ID: wpr-228411
ABSTRACT
Goltz syndrome or focal dermal hypoplasia, is a rare genodermatosis, characterized by multiple abnormalities of mesodemal and ectodermal organs. Cutaneous manifestations include linear or cribriform atrophied patches with telangiectasia following Blaschko line and papillomas. The majority of patients also have skeletal abnormalities, such as syndactyly, oligodactyly, scoliosis, and short stature. Furthermore, various ophthalmic and dental manifestations have been reported. We report a case of Goltz syndrome occurring in a 2-year old female with asymptomatic erythematous atrophic patches with purpuric macules and telangiectasia mainly on extremities. She also had hypotrichia on vertex and a notching-like cleft on upper lip. A skin biopsy performed on her atrophic patch on the leg revealed dermal hypoplasia. The final diagnosis was made by typical cutaneous manifestations and histopathological findings compatible with focal dermal hypoplasia.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Papilloma
/
Scoliosis
/
Skin
/
Telangiectasis
/
Abnormalities, Multiple
/
Biopsy
/
Focal Dermal Hypoplasia
/
Syndactyly
/
Ectoderm
/
Extremities
Limits:
Female
/
Humans
Language:
Korean
Journal:
Korean Journal of Dermatology
Year:
2008
Type:
Article
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