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November 1982

Familial Erythromelalgia

Author Affiliations

From the Division of Dermatology, University of Maryland School of Medicine, Baltimore.

Arch Dermatol. 1982;118(11):953-954. doi:10.1001/archderm.1982.01650230081037

Erythromelalgia is a rare disorder characterized by erythema, burning, and increased skin temperature of the extremities. Both primary and secondary forms of this entity have been described.1

Primary erythromelalgia may be transmitted as a dominant genetic disease. Reports of two such kindreds have appeared in the English literature.2,3 Members of another kindred with erythromelalgia seemed to have inherited the disorder as a dominant trait with partial sex linkage. The onset of symptoms in these persons occurred at puberty, and their disease was later accompanied by nephritis.4 It is not clear whether this family's erythromelalgia was primary or secondary to the renal disease. In this report, we describe a third kindred in which two siblings had the primary form of the disease. No other family members were affected.

Report of Cases 

Case 1.—  A 20-year-old man was first examined at the University of Maryland Hospital, Baltimore, in February

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