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At a glance

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

Synonyms

Gorlin Bushkell Jensen Syndrome; Porcelain Nails Disease.

Incidence

The exact incidence remains unknown. It is expected to be significant worldwide as it is often associated with other medical conditions, either acquired or congenital.

Genetic inheritance

It is suggested as an autosomal recessive inheritance pattern.

Pathophysiology

Incomplete keratinization in the intermediate part of the nail plate.

Clinical aspects

Clinical presence of completely white nails, together with histologic evidence of a defect in the nail plate. Leukonychia totalis, koilonychia, multiple sebaceous cysts, and renal calculi.

Precautions before anesthesia

There are no specific precautions before anesthesia except for the possibility of an underlying medical condition that could carry its own potential considerations.

Anesthetic considerations

The perioperative considerations are determined by the underlying medical conditions/intoxications. Patients receiving chronic corticosteroid therapy must be carefully prepared and intraoperative supplementation might be required.

Other condition to be considered

See “Leukonychia Overview” and Table L-2 for completion.

References

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Canavan  T, Tosti  A, Mallory  H, McKay  K, Cantrell  W, Elewski  B: An idiopathic leukonychia totalis and leukonychia partialis case report and review of the literature. Skin Appendage Disord 1(1):38–42, 2015.  [PubMed: 27172289]
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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.  [PubMed: 11174421]
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Frydman  M, Cohen  HA: Leukonychia totalis in two sibs. Am J Med Genet 47:540, 1993.  [PubMed: 8256820]

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