Bloch-sulzberger Syndrome (incontinentia Pigmenti): a Case Report
نویسنده
چکیده
Incontinentia pigmenti (IP) is a X-linked dominant neuro cutaneous syndrome with cutaneous, neurologic, ophthalmologic and dental manifestations mainly in female neonates. Starting from neonatal period, IP passes through stages of vesicular (90%), verrucous (70%), hyper pigmented (98%) and hypopigmented lesions. Authors report a case of IP in 8 months old female child who presented with vesiculo bullous skin eruption and verrucous lesions on hand and feet. The diagnosis was confirmed on histopathology. Variable pattern of lesions presenting over a period of time makes it difficult to diagnose. As disease is incurable, genetic counseling is of paramount importance.
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 (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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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 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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