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Genetically transmitted anomaly of nails (presence of white nails).

Gorlin Bushkell Jensen Syndrome; Porcelain Nails Disease.

Incidence is unknown. Inherited as an autosomal dominant; however, an autosomal recessive inheritance as been suggested.

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 be required.

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 47:540, 1993.  [PubMed: 8256820]

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