Table of Contents
ISRN Dermatology
Volume 2011, Article ID 814186, 3 pages
Case Report

Incontinentia Pigmenti and Bipolar Aphthosis: An Unusual Combination

1Servicio Dermatología, Hospital Universitari Sagrat Cor, 08029 Barcelona, Spain
2Servicio Dermatología, Hospital Sant Joan de Déu Barcelona, 08950 Barcelona, Spain
3Servicio Reumatología, Hospital Sant Joan de Déu Barcelona, 08950 Barcelona, Spain

Received 15 November 2010; Accepted 6 January 2011

Academic Editors: A. R. Ahmed and C. Huh

Copyright © 2011 G. Márquez Balbás et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Incontinentia pigmenti (IP) is an uncommon X-linked dominant multisystem disorder, lethal in the majority of affected males in utero and variably expressed in females. The cutaneous manifestations are diagnostic and classically occur in four stages: vesicular, verrucous, hyperpigmented, and atrophic. The skin lesions are typically spread along the lines of Blaschko, and they are usually present at birth. It may be variably accompanied by dental, ocular, neurologic, bones and joints, and development anomalies. The genes IP has been mapped to Xq28. Mutations in the NEMO/IKKγ gene, located at Xq28, have been found to cause expression of the disease. Behçets disease is a multisystem disorder consisting of recurrent oral aphtae, genital ulcers, pustular skin eruption, and uveitis. Occasionally there are other articular, neurological, intestinal, or vascular abnormalities. This disease is rare in children. Here, we report a case of a 16-year-old female with the rare combination of incontinentia pigmenti and an aphthosis bipolar, and we discuss the probably relationship between these two diseases.