This Article  • References  • Full text PDF  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citation map  • Citing articles on HighWire  • Citing articles on Web of Science (46)  • Contact me when this article is cited  Related Content  • Similar articles in this journal  Social Bookmarking   What's this?

HELEN O. CURTH, MD; DOROTHY WARBURTON, PhD

Arch Dermatol. 1965;92(3):229-235.

Abstract
The familial incidence of incontinentia pigmenti and its almost exclusive occurrence in females have been explained by two theories. One theory (Pfeiffer³⁶) suggests that the disease is due to an autosomal, dominant gene, which is sex-limited in its expression, producing the defect only in females. The other theory (Lenz³) suggests that incontinentia pigmenti is due to a sex-linked gene carried on the X-chromosome. This gene acts as a dominant in females and is lethal in males. The pros and cons of each theory are discussed.

The Franceschetti-Jadassohn type of incontinentia pigmenti is clinically and genetically different from the classic type. A third of "transitional" cases occurs in boys and girls. Its genetic mechanism has not yet been determined.

Author Affiliations
NEW YORK

From the Department of Dermatology, and the Department of Obstetrics and Gynecology, College of Physicians and Surgeons, Columbia University.

Footnotes
Accepted for publication May 14, 1965.

Paper read at the December 1964 Symposium of the Dermatologic Society of Greater New York (formerly the Bronx Dermatological Society) on Genodermatoses.

Reprint requests to 35 East 84th St, New York, NY 10028 (Dr. Curth).

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter     What's this?

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

Incontinentia Pigmenti: A Four-Generation Study
Wiklund and Weston
Arch Dermatol 1980;116:701-703.
ABSTRACT  

Zebra-like Hyperpigmentation in an Infant With Multiple Congenital Defects
Alimurung et al.
Arch Dermatol 1979;115:878-881.
ABSTRACT  

Incontinentia Pigmenti: A World Statistical Analysis
Carney
Arch Dermatol 1976;112:535-542.
ABSTRACT  

Depigmented Lesions in Incontinentia Pigmenti: A Useful Diagnostic Sign
Wiley and Frias
Arch Pediatr Adolesc Med 1974;128:546-547.
ABSTRACT  

Incontinentia Pigmenti: A Systemic Genodermatosis with Striking Cutaneous Findings
Gurevitch et al.
CLIN PEDIATR 1973;12:396-401.
ABSTRACT  

Incontinentia Pigmenti (Bloch-Sulzberger Syndrome) A Report of Four Additional Cases
Morgan
Arch Pediatr Adolesc Med 1971;122:294-300.
ABSTRACT  

Hereditary Acrokeratotic Poikiloderma
Weary et al.
Arch Dermatol 1971;103:409-422.
ABSTRACT  

Incontinentia Pigmenti (Bloch-Sulzberger Syndrome): Report of a Negro Infant wiih Typical Skin Lesions, Alopecia, and Delayed Atypical Dentition
Surana and Scott
CLIN PEDIATR 1969;8:286-289.
 

Incontinentia Pigmenti Associated With Subungual Tumors
HARTMAN
Arch Dermatol 1966;94:632-635.
ABSTRACT