Retinal Neovascularization in Two Patients with Incontinentia Pigmenti

被引：0

作者：

Dwiyana, Reiva Farah ^{[1
]}

Banjarnahor, Ivan Daniel ^{[1
]}

Diana, Inne Arline ^{[1
]}

Gondokaryono, Srie Prihianti ^{[1
]}

Effendi, Raden Mohamad Rendy Ariezal ^{[1
]}

Feriza, Vina ^{[1
]}

机构：

[1] Univ Padjadjaran Dr Hasan Sadikin Hosp, Fac Med, Dept Dermatol & Venereol, Bandung, Indonesia

来源：

CLINICAL COSMETIC AND INVESTIGATIONAL DERMATOLOGY | 2022年 / 15卷

关键词：

genodermatosis; incontinentia pigmenti; ocular manifestation; retina; MANIFESTATIONS;

D O I：

10.2147/CCID.S363179

中图分类号：

R75 [皮肤病学与性病学];

学科分类号：

100206 ;

摘要：

Incontinentia pigmenti (IP) is a rare genodermatosis, inherited in an X-linked dominant pattern, making it generally found among women. Among several characteristics of IP are four phases of skin manifestation that tend to follow Blaschko's lines, in addition to abnormalities of the eye, central nervous system (CNS), and teeth. Ocular involvement in IP patients can occur since birth, which can be classified into retinal or non-retinal disorders. Retinal disorders can result in detachment, which is a major ocular threat for IP patients. This article reports two IP cases with overlapped phases of skin disorders in baby girls with ocular manifestations since early life. Clinical signs and additional examination of the skin and eyes are utilized to make the diagnosis. All the features of the histopathological examination supported the diagnosis of IP, and ocular exams revealed abnormalities in the form of retinal neovascularization (RN). Although RN may resolve spontaneously, patients should be monitored for the development of other eye disorders such as visual impairment.

引用

页码：803 / 808

页数：6

共 18 条

[1] Incontinentia pigmenti: A review and update on the molecular basis of pathophysiology [J].