A Case of Incontinentia Pigmenti Associated with Multiorgan Abnormalities

Chung, Woon-Kyong; Lee, Deokwoo; Chang, Sung‐Eun; Lee, Mi-Woo; Choi, Jee‐Ho; Moon, Kee‐Chan

doi:10.5021/ad.2009.21.1.56

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Cited by 3 publications

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“…It is a rare heritable multisystem ectodermal disorder that has dermatologic, dental, ocular and neurologic abnormalities. 1,2…”

Section: Introductionmentioning

confidence: 99%

Bloch-Sulzberger syndrome: a rare genodermatosis

Thomas

2023

Int J Contemp Pediatr

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A 6 month old female child was admitted with complaints of delayed developmental milestones and multiple hyperpigmented patches on the trunk, limbs, axilla and groin. At birth the baby had diffuse vesciculo-bullous rash. Currently the child has global developmental delay with seizures with investigations suggesting the same. Clinically the child was diagnosed as a case of Incontinentia pigmenti. Early identification of this condition with a multi disiplinary approach is associated with better outcomes.

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“…It is a rare heritable multisystem ectodermal disorder that has dermatologic, dental, ocular and neurologic abnormalities. 1,2…”

Section: Introductionmentioning

confidence: 99%

Bloch-Sulzberger syndrome: a rare genodermatosis

Thomas

2023

Int J Contemp Pediatr

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“…First described by Bloch in 1926, and Sulzberger in 1928, incontinentia pigmenti (IP) is a rare X-linked genodermatosis [ 1 , 2 ], which name is related to the histological characteristics of the lesions in the third stage (or pigmentary stage) of the disease ( Tab.1 ), consisting in the melanin incontinence by melanocytes of the basal epidermal layer and by its presence in the superficial dermis.…”

Section: Introductionmentioning

confidence: 99%