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At a glance

It is a congenital palmoplantar keratoderma and nail dystrophy syndrome. Patient presents with progressive perioral and perineal keratoderma associated with bilateral corneal epithelial dysplasia leading to severe corneal scarring and impairment of vision.

Synonym

Palmoplantar Periorofacial Keratoderma Corneal Epithelial Dysplasia Syndrome.

Genetic inheritance

Unknown.

Clinical aspects

Dermatologic signs are very common: dry skin, palmoplantar hyperkeratosis, abnormal fingernails, and generalized absence of hair. Involvement of the corneas is common, possibly resulting in visual impairment.

Anesthetic considerations

No reports found, but possible concerns include difficulty placing and securing monitoring equipment (adhesive ECG lead pads) and indwelling lines (IV catheters, arterial lines). Hyperkeratosis, if severe, could possibly limit joint mobility, resulting in contractures, which may make positioning the patient more awkward. Care must be taken to maintain the natural position of the patient and to pad all pressure points well. Difficult airway management might be expected if mouth opening or neck mobility is limited because of contractures.

References

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Judge  MR, Misch  K, Wright  P,  et al: Palmoplantar and periorofacial keratoderma with corneal epithelial dysplasia: A new syndrome. Br J Dermatol 125:186, 1991.  [PubMed: 1832929]

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