Description of the disease * EnglishPolish Pobierz sekcję do PDF Definition 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. Disease data Classification Disease Synonyms Bronspiegel-Zelnick syndrome Autosomalna recesywna aplazja skóry Zespół Bronspiegela i Zelnicka ORPHA code 1116 OMIM code 207731 ICD10 code Q84.8 ICD11 code LD27.Y *Soruce Extended description of the disease Pobierz sekcję do PDF No additional description. Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl