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

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Gorlin Bushkell Jensen Syndrome; Porcelain Nails Disease.

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

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Incomplete keratinization in the intermediate part of the nail plate.

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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.

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No specific precautions before anesthesia and perioperative considerations except for patients receiving chronic corticosteroid therapy, in whom intraoperative supplementation might be required.

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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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