Knuckle Pads, Leukonychia and Deafness

THE present report concerns a family in which knuckle pads, mixed hearing loss (sensorineural and conductive) and total leukonychia (white nails) are inherited together, following the principles of dominant mendelian genetics. To our knowledge, this hereditary syndrome has not previously been reported.Case ReportsCase 1. The proposita, E.J.H., an 11-year-old girl, 1 of 4 siblings, was first seen because of a mild pyoderma and possible atopic dermatitis of the upper extremities, both of which responded to topical therapy. It was noted that she had firm, though not markedly hyperkeratotic, thickened skin over almost all the interphalangeal joints of the . . .