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Case Reports
. 2009 Feb;21(1):56-9.
doi: 10.5021/ad.2009.21.1.56. Epub 2009 Feb 28.

A case of incontinentia pigmenti associated with multiorgan abnormalities

Woon-Kyong Chung  1 , Deok-Woo Lee, Sung-Eun Chang, Mi-Woo Lee, Jee-Ho Choi, Kee-Chan Moon
Affiliations
Case Reports

A case of incontinentia pigmenti associated with multiorgan abnormalities

Woon-Kyong Chung et al. Ann Dermatol. 2009 Feb.

Abstract

Incontinentia pigmenti is a systemic disorder affecting the skin, teeth, eyes, nervous tissue, hair, nails, musculoskeletal system, and heart. We describe an 11-month-old girl with incontinentia pigmenti associated with a ventricular septal defect, left hemiatrophy, hemangiomas, an abnormal labial frenum, and spastic cerebral palsy manifested as left hemiplegia and developmental delay. We believe this patient illustrates that incontinentia pigmenti is a systemic disorder necessitating a multidisciplinary approach to management.

Keywords: Genodermatosis; Incontinentia pigmenti; Systemic disorder.

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Figures

Fig. 1
Fig. 1
Linear and whorled brownish patches on the trunk (A) and extremities (B), reddish plaque on the back (C).
Fig. 2
Fig. 2
A large perimembranous ventricular septal defect is seen on echocardiography.
Fig. 3
Fig. 3
Histopathologically, focal vacuolar change of the basal layer (A) and a dermal melanophage are observed (B) (H&E, ×400).
Fig. 4
Fig. 4
At the age of 14 months, Bell Thomson study shows that the right leg is 1.4 cm longer than the left leg.
Fig. 5
Fig. 5
Abnormal labial frenum.
See this image and copyright information in PMC

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