Gingival fibromatosis-hypertrichosis syndrome

Orpha code: 2026OMIM code: 135400

Definition

A rare autosomal dominant disorder characterized by a generalized enlargement of the gingiva occurring at birth or during childhood that is associated with generalized hypertrichosis developing at birth, during the first years of life, or at puberty and predominantly affecting the face, upper limbs, and midback.

Disease data
Classification

Malformation syndrome

Synonyms
CGHT
CGHT
Hipertrichoza z lub bez hiperplazji dziąseł
Wrodzona uogólniona hipertrichoza graniczna
Zespół hirsutyzmu i wrodzonego przerostu dziąseł
Congenital generalized hypertrichosis terminalis
Hirsutism-congenital gingival hyperplasia syndrome
Hypertrichosis with or without gingival hyperplasia
ORPHA code
2026
OMIM code
135400
ICD10 code
L68.8
ICD11 code
LD27.0Y

No additional description.

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