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Article
May 1977

Pachyonychia Congenita With Recessive Inheritance

Author Affiliations

Republic of Singapore

Kuala Lumpur, West Malaysia

Arch Dermatol. 1977;113(5):685-686. doi:10.1001/archderm.1977.01640050145033
Abstract

To the Editor.—  Pachyonychia congenita (PC), a rare genodermatosis, to our knowledge was first described by Jadassohn and Lewandowsky in 1906. It is characterized by the typically thickened dystrophic nails, keratoderma of the palms and soles, hyperhidrosis, plantar blisters, keratosis pilaris, leukokeratosis oris, ichythosis, steatocytoma multiplex, epidermal cysts, and other features of dyskeratosis such as corneal dystrophy, cataract, hypoplastic hair, and changes in the teeth, nose, ears, and mucous membranes.A majority of the cases were reported from Europe and America, and studies from several large families have demonstrated that PC is transmitted as a simple mendelian dominant gene with incomplete penetrance.2-6Asian cases were rarely reported, and the disease, as far as we are aware, has not been known to be inherited as an autosomal recessive trait.Recently, an Indian girl from Western Malaysia was found to have PC, but the disease appeared to be a recessive disorder, as she was

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