X-linked incontinentia pigmenti or Bloch-Sulzberger syndrome: a case report.
نویسندگان
چکیده
Incontinentia pigmenti is a rare X-linked genodermatosis that affects mainly female neonates. Skin manifestations are the most common and occur in four quite distinct phases. A female infant presented vesiculobullous lesions on trunk and limbs, and a verrucous lesion on the right palm. Biopsy revealed eosinophil exocytosis and pigment incontinence, confirming the clinical hypothesis. Although uncommon, incontinentia pigmenti should be taken into consideration as a possible differential diagnosis when vesiculobullous and verrucous lesions are present in childhood.
منابع مشابه
Incontinenta pigmenti (Bloch-Sulzberger disease) در یک دختر 10 ساله
Abstract Incontinenta pigmenti or Bloch-Sulzberger syndrome is a rare x-linked dominant disease that mainly affects the skin, eye, hair, central nervous system and teeth. The disease is predominant among women. Dermatologic manifestations are among the most important aspects for the diagnosis of the syndrome. Oral involvement characterized by hypodontia of deciduous and permanent teeth is i...
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Background: Incontinentia pigmenti (IP), also known as Bloch-Sulzberger syndrome, is a rare X-linked dominant genodermatosis that presents at the time of birth or soon after birth with cutaneous manifestation. This disorder may also affect the ectodermal tissues, such as the central nervous system, skeletal system, eyes, hair, nails, and teeth. The dermatological findings occur in four successi...
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Incontinentia pigmenti is a rare X-linked genodermatosis that affects mainly female neonates. Skin manifestations are the most common and occur in four quite distinct phases. A female infant presented vesiculobullous lesions on trunk and limbs, and a verrucous lesion on the right palm. Biopsy revealed eosinophil exocytosis and pigment incontinence, confirming the clinical hypothesis. Although u...
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Incontinentia pigmenti (IP, Bloch-Sulzberger syndrome) is a very rare genodermatosis characterized by typical skin lesions accompanied by dental, central nervous system, bone and ocular abnormalities. Incontinentia pigmenti is usually observed among women, as this X-linked dominantly inherited disorder is lethal in males. The hallmark feature of IP is cutaneous eruption along the lines of Blasc...
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ورودعنوان ژورنال:
- Anais brasileiros de dermatologia
دوره 85 3 شماره
صفحات -
تاریخ انتشار 2010