Aplasia cutis congenita	Autosomal recessive hereditary disorder	Congenital anomaly of lower trunk	Congenital anomaly of lymphatic structure of trunk	Congenital lymphangiectasia	Developmental hereditary disorder	Hereditary disorder of lymphatic system	Hereditary disorder of the integument	Intestinal lymphangiectasis
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Aplasia cutis congenita with intestinal lymphangiectasia syndrome (disorder)
	Aplasia cutis congenita with intestinal lymphangiectasia syndrome
	Bronspiegel Zelnick syndrome
	An extremely rare association syndrome, described in only two brothers to date (one of which died at 2 months of age), characterized by aplasia cutis congenita of the vertex and generalized edema (as well as hypoproteinemia and lymphopenia) due to intestinal lymphangiectasia. There have been no further descriptions in the literature since 1985.
	syndroom van aplasia cutis congenita met intestinale lymfangiëctasie
	syndroom van Bronspiegel-Zelnick Bronspiegel-Zelnick-syndroom

Id	720500008
Status	Primitive

Associated morphology	Aplasia
Finding site	Skin part
Occurrence	Congenital
Pathological process	Pathological developmental process

Associated morphology	Lymphangiectasis
Finding site	Structure of lymphatic vessel of intestine
Occurrence	Congenital
Pathological process	Pathological developmental process

DHD Diagnosis thesaurus reference set

ICD-10 complex map reference set

Target	Q84.8
Rule	TRUE
Advice	ALWAYS Q84.8
Correlation	SNOMED CT source code to target map code correlation not specified

Target	Q82.0
Rule	TRUE
Advice	ALWAYS Q82.0
Correlation	SNOMED CT source code to target map code correlation not specified