Preferred Label : Aplasia cutis congenita - intestinal lymphangiectasia;
ICD-11 definition : Aplasia cutis congenita - intestinal lymphangiectasia refers to the association of
aplasia cutis congenita, a rare skin defect usually localized to the vertex and intestinal
lymphangiectasia, presenting with generalized edema and hypoproteinemia and lymphopenia.;
ICD-11 synonym : Autosomal recessive aplasia cutis; Bronspiegel-Zelnick syndrome;
Origin ID : 1746934910;
Currated CISMeF NLP mapping
Aplasia cutis congenita - intestinal lymphangiectasia refers to the association of
aplasia cutis congenita, a rare skin defect usually localized to the vertex and intestinal
lymphangiectasia, presenting with generalized edema and hypoproteinemia and lymphopenia.
