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Pachyonychia Congenita

Am J Dis Child. 1966;111(6):649-652. doi:10.1001/archpedi.1966.02090090121014.
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PACHYONYCHIA congenita is an ectodermal defect in which marked thickening of the nails of all extremities, beginning early in life, is a constant feature. Skin bullae and mucous membrane abnormalities are also part of the syndrome. Plugging of the skin follicles is often present.1

Report of a Case  The patient, a Negro boy of 15 months of age, was seen at the Child Evaluation Center because of growth failure and possible mental retardation. The referring agency, Maricopa County General Hospital, is well acquainted with the child's mother. She has been treated there for sores on the bottoms of her feet, thick scaly skin on her heels, keratotic lesions of the face, and ichthyosis involving several areas of the body as well as nail thickness (Fig 1). The mother's walk at times is almost a hobble. Treatment has included 5% salicylic acid in a water-miscible ointment base (Aquaphor) to the


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