Genetically transmitted anomaly of nails (presence of
Gorlin Bushkell Jensen Syndrome; Porcelain Nails Disease.
Incidence is unknown. Inherited
as an autosomal dominant; however, an autosomal recessive inheritance as
Incomplete keratinization in the intermediate part
of the nail plate.
Clinical presence of completely white nails,
together with histologic evidence of a defect in the nail plate. Leukonychia
totalis, koilonychia, multiple sebaceous cysts, renal calculi.
No specific precautions before
anesthesia and perioperative considerations except for patients receiving
chronic corticosteroid therapy, in whom intraoperative supplementation might
See Table L-2 comparing
leukonychia and other nail discoloration syndromes.
Claudel CD, Zic JA, Boyd AS: Idiopathic leukonychia totalis and partialis
in a 12-year-old patient. J Am Acad Dermatol 44(suppl):379, 2001.
Frydman M, Cohen HA: Leukonychia totalis in two sibs. Am J Med Genet