True about incontinenta pigmenti include the following except –
Correct Answer: Ocular involvement is seen in almost 100% cases and is typically unilateral
Description: Incontinentia pigmenti (IP) is a rare X-linked dominant genetic disorder that affects the skin, hair, teeth, nails and central nervous system. It is named from its appearance under a microscope. The cardinal feature is the appearance of characteristic progressive skin lesions, first presenting as vesiculobullous lesions and then progressing to whorl-like pigmentary lesions over four stages. IP also includes eye abnormalities, mostly occurring in the retina. The retinal findings stem from vascular occlusion and include neovascularization, hemorrhages, absence of foveal pit, and exudative and tractional detachments. Pathologic changes in the central nervous system, teeth, and hair are also common in IP. Diagnosis is based on clinical systemic and ocular exam, as well as genetic testing. There is no treatment for the disease as a whole; however, symptoms may be managed by medical or surgical intervention. Ref Harrison20th edition pg 1456
Category:
Dental
Get More
Subject Mock Tests
Practice with over 200,000 questions from various medical subjects and improve your knowledge.
Attempt a mock test nowMock Exam
Take an exam with 100 random questions selected from all subjects to test your knowledge.
Coming SoonGet More
Subject Mock Tests
Try practicing mock tests with over 200,000 questions from various medical subjects.
Attempt a mock test now