Aplasia cutis congenita intestinal lymphangiectasia
Other Names: ACC with intestinal lymphangiectasia
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.
NIH genetic and rare disease info[edit source]
Aplasia cutis congenita intestinal lymphangiectasia is a rare disease.
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