A Case of Hunter Syndrome / 대한피부과학회지
Korean Journal of Dermatology
; : 928-932, 2008.
Article
en Ko
| WPRIM
| ID: wpr-78584
Biblioteca responsable:
WPRO
ABSTRACT
We report a case of Hunter syndrome in a 4 year old boy, who presented with firm skin colored papules and nodules that coalesce to form a reticular pattern (pebbling of the skin) with extensive Mongolian spots. The lesions are arranged bilaterally and symmetrically over the scapulae, upper arm and lateral aspects of the thighs. He also has low intelligence, coarse face, saddle nose and claw hand contracture of both hands. The result of qualitative analysis of urine was positive for dermatan sulfate and heparan sulfate. And enzyme activity of iduronate-2-sulfatase is decreased in plasma and leukocyte. A skin biopsy specimen section stained with hematoxylin-eosin showed widely separated collagen bundles in the dermis associated with mucin deposition.
Palabras clave
Texto completo:
1
Índice:
WPRIM
Asunto principal:
Brazo
/
Plasma
/
Escápula
/
Piel
/
Muslo
/
Biopsia
/
Nariz
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Colágeno
/
Mucopolisacaridosis II
/
Contractura
Tipo de estudio:
Qualitative_research
Límite:
Animals
Idioma:
Ko
Revista:
Korean Journal of Dermatology
Año:
2008
Tipo del documento:
Article