Preferred Label : Singleton-Merten dysplasia;
ICD-11 definition : Singleton-Merten dysplasia is characterized by dental dysplasia, progressive calcification
of the thoracic aorta with stenosis, osteoporosis and expansion of the marrow cavities
in hand bones. Additional features included generalized muscle weakness and atrophy,
and chronic psoriasiform skin eruptions.;
Origin ID : 1084593684;
Automatic exact mappings (from CISMeF team)
Singleton-Merten dysplasia is characterized by dental dysplasia, progressive calcification
of the thoracic aorta with stenosis, osteoporosis and expansion of the marrow cavities
in hand bones. Additional features included generalized muscle weakness and atrophy,
and chronic psoriasiform skin eruptions.