The scalp-nipple-ear syndrome (SENS) is a rare hereditary condition with a pattern of skin involvement.

Inheritance: autosomal dominant


Key clinical features:

(1) aplasia cutis congenita of the scalp (with or without lumpy scalp)

(2) ear malformations (cupped, folded, etc)

(3) rudimentary nipples (hypothelia)


Additional findings may include:

(1) renal insufficiency

(2) cataract

(3) dental changes (widely spaced or missing secondary teeth)

(4) syndactyly

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