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August 1986

Autosomal Recessive Pachyonychia Congenita

Author Affiliations

From the Division of Dermatology, Toronto General Hospital (Dr Haber), and the Department of Pathology, Women's College Hospital (Dr Rose), University of Toronto.

Arch Dermatol. 1986;122(8):919-923. doi:10.1001/archderm.1986.01660200091023

• We report the second and third cases of pachyonychia congenita inherited as an autosomal recessive disorder. Our cases were unusual, with the fingernails showing a striking leukonychia and appearing clinically as Terry's nails. These patients were originally diagnosed as having epidermolysis bullosa simplex because of a history of a life-long blistering disorder. The clinical features and inheritance of pachyonychia congenita, as well as the reasons for the long delay in diagnosis of our cases, are discussed.

(Arch Dermatol 1986;122:919-923)

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