The basal cell nevus syndrome is hereditary with multiple defects involving organs and systems of both ectodermal and mesodermal origin. While the major components of the syndrome (basal cell epitheliomas, mandibular cysts, minor rib anomalies) are well recognized, there are a number of less constant defects which merit attention and further study. Some of these defects suggest a relationship between the basal cell nevus syndrome and certain other rare hereditary or congenital syndromes which have hitherto been considered unrelated.
CLENDENNING WE, BLOCK JB, RADDE IC. Basal Cell Nevus Syndrome. Arch Dermatol. 1964;90(1):38–53. doi:10.1001/archderm.1964.01600010044011
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