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October 1974

Classification of the Sclerodermoid Genodermatoses

Author Affiliations

Burbank, Calif

Arch Dermatol. 1974;110(4):641. doi:10.1001/archderm.1974.01630100089036

To the Editor.—  Scleroderma in all of its forms is a disease pattern of multiple origins. There are thirteen genetic conditions that cause scleroderma of the skin. A study of these diseases may provide clues to the pathogenics of systemic scleroderma.The sclerodermoid genodermatoses can be divided into metabolic, heredodegenerative, and miscellaneous disorders; these sclerodermoid changes and their mode of inheritance1 are as follows:Metabolic disorder Erythropoietic porphyria—autosomal recessive Erythropoietic porphyria—autosomal dominant Metabolic disorder Erythropoietic porphyria—autosomal recessive Erythorhepatic porphyria—autosomal dominant Variegate porphyria—autosomal dominant Porphyria cutanea tarda—autosomal dominant (?) Phenylketonuria—autosomal recessive (?) Muscular glycogenesis of unknown type7 Heredodegenerative disorders Werner Syndrome—autosomal recessive Ataxia Telangiectasia—autosomal recessive Rothmund syndrome—autosomal recessive Winchester syndrome—autosomal recessive Progeria—autosomal recessive (?) Others Epidermolysis bullosa dystrophica— autosomal dominant and recessive Stiff skin syndrome—autosomal dominantThe largest group of metabolic disorders are the porphyrias, in which the skin is scarred by the photosensitivity from the porphyrins.2,3 In all these disorders