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April 1961

Ichthyosis Linearis Circumflexa: A Variant of Congenital Ichthyosiform Erythroderma

Author Affiliations


From the Dermatology Service, Walter Reed General Hospital, WRAMC (Col. Robert S. Higdon, MC, USA, Chief of Service), and the Department of Dermatology, Walter Reed Army Institute of Research, WRAMC, Washington, D.C.

Arch Dermatol. 1961;83(4):630-635. doi:10.1001/archderm.1961.01580100094012

The recent observation of 2 children with a bizarre, annular, and polycyclic keratotic dermatosis of a migratory nature prompted us to review the medical literature in an effort to locate similar cases. A report by Stevanovic1 was encountered in which he entitled a comparable eruption "dyskeratosis ichthyosiformis congenita migrans." Reference was given in Stevanovic's article to other cases reported in the European literature.2-5 Priority for recognition of this disorder as a previously undescribed dermatosis belongs to Comel,2 who reported the original example under the designation "ichthyosis linearis circumflexa." To the best of our knowledge, our cases are the first to have occurred in siblings and the first to have been described from the United States. We wish to review those cases previously reported and to add these additional cases to the literature.

Report of Cases 

Case 1.—  A 6-year-old girl, only sibling of Case 2, was originally seen