Preferred Label : Lethal restrictive dermopathy;
ICD-11 definition : Lethal tight skin contracture syndrome is an inherited syndrome characterized by the
skin being drawn tightly over the face causing a narrow, pinched nose, small mouth,
limited jaw mobility, and entropion.Severe and constant intrauterine growth retardation
is observed as well as multiple joint contractures, and rigid and tense skin is easily
eroded with fissures in the axilla and neck. The major manifestations are the result
of restricted in utero movements secondary to defective skin.;
Origin ID : 713433700;
Currated CISMeF NLP mapping
- Validated automatic mappings to NTBT
Lethal tight skin contracture syndrome is an inherited syndrome characterized by the
skin being drawn tightly over the face causing a narrow, pinched nose, small mouth,
limited jaw mobility, and entropion.Severe and constant intrauterine growth retardation
is observed as well as multiple joint contractures, and rigid and tense skin is easily
eroded with fissures in the axilla and neck. The major manifestations are the result
of restricted in utero movements secondary to defective skin.
