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March 1962

Pachyonychia Congenita

Author Affiliations


Clinical Instructor in Medicine (Dermatology), The University of Rochester School of Medicine and Dentistry; Assistant Physician, Strong Memorial Hospital (Dr. Buckley); A.E.C. Fellow in Occupational Medicine, The University of Rochester School of Medicine and Dentistry (Dr. Cassuto).

Arch Dermatol. 1962;85(3):397-402. doi:10.1001/archderm.1962.01590030095013

Pachyonychia congenita is a rarely reported congenital ectodermal defect characterized by deformity of the nails, leukokeratosis of mucous membranes, and cutaneous changes in which pigmentation may be a prominent feature.1-3 Although there is still some controversy as to the genetic transmission of the disease, the defect is presumably transmitted as a dominant and seldom continues for more than 2 generations.4 This particular case presented a somewhat milder variant of the disease with pigmentary changes being a most prominent finding.

Report of a Case  A 23-year-old white male of Swedish ancestry was observed on routine examination to have widespread pigmentary changes and deformed nails. He advised that these changes had been present as long as he could remember—at least, during very early childhood. Both parents had died before the patient reached one year of age. His physical and mental growth had been normal; his general health was excellent. No