Linear skin atrophy, scarring alopecia, anonychia, and tongue lesion: A “new” syndrome?
Article first published online: 3 JUN 2005
Copyright © 1985 Wiley-Liss, Inc., A Wiley Company
American Journal of Medical Genetics
Volume 21, Issue 4, pages 669–680, August 1985
How to Cite
Sequeiros, J., Sack, G. H., Opitz, J. M. and Reynolds, J. F. (1985), Linear skin atrophy, scarring alopecia, anonychia, and tongue lesion: A “new” syndrome?. Am. J. Med. Genet., 21: 669–680. doi: 10.1002/ajmg.1320210408
- Issue published online: 3 JUN 2005
- Article first published online: 3 JUN 2005
- Manuscript Revised: 28 JAN 1985
- Manuscript Received: 30 APR 1984
- skin atrophy;
- congenital alopecia;
- MZ twin discordance;
- alopecia areata
One of a pair of female monozygotic twins showed skin atrophy with linear alternation of depressed scarlike areas and intervening ridges of normal or nearly normal skin. She was born with friable skin and a vesicular-bullous eruption which was followed by gradual scabbing. Hypohidrosis in the affected areas, heat intolerance, and febrile convulsions were noted in infancy and childhood. No new skin lesions developed, and the existing ones, the sweating disturbance, and the heat intolerance gradually improved with time. Scarring alopecia, congenital absence of three toenails, and a scarlike lesion of the tongue were also present. Their absence in the other twin supports the view that 1) these manifestations all are part of the same syndrome, and 2) this syndrome is nongenetic.
Histologically, there were no diagnostic or consistent findings, but the number of skin appendages was diminished, and the elastic fibers were reduced in number and size in one biopsy. The calculated probability for the twins being monozygotic was 0.9998.
This family was also remarkable for the presence of alopecia areata in three successive generations with only one instance of apparent nonpenetrance.
We conclude that this may represent a previously undescribed syndrome of congenital fragility of connective tissue which predisposed to damage of the elastica, possibly caused by an early inflammatory phase.