Congenital palmoplantar keratoderma and nail dystrophy.
Patient presents with progressive perioral and perineal keratoderma associated with
bilateral corneal epithelial dysplasia leading to severe corneal scarring
and impairment of vision.
Palmoplantar and Periorofacial Keratoderma with Corneal
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
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.
Judge MR, Misch K, Wright P, et al: Palmoplantar and periorofacial
keratoderma with corneal epithelial dysplasia: A new syndrome. Br J Dermatol