A Case Report of a Bloch-sulzberger Syndrome (Incontinentia Pigmenti) Patient on Whom We Performed Orthognathic Surgery under General Anesthesia
نویسندگان
چکیده
منابع مشابه
Bloch-sulzberger Syndrome (incontinentia Pigmenti): a Case Report
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 vesicul...
متن کامل[Incontinentia pigmenti (Bloch-Sulzberger syndrome)].
Incontinentia pigmenti (Bloch-Sulzberger syndrome) is a genetic disease of the skin with generalised ectodermal and mesodermal dysplasia which may often involve the eyes (35% of the patients),' hair, teeth, and central nervous system. Skin lesions are Correspondence to Dr A Spallone. usually present at birth or shortly after in the form of erythematous eruptions with linear vesiculations. The f...
متن کامل[Incontinentia pigmenti (the Bloch-Sulzberger syndrome)].
The course and histologic skin signs are described at the stage of inflammation in 2 cases of incontinentia pigmenti (IP) or the Bloch-Sulzberger syndrome. In the authors' opinion, the pigmentary stage of skin lesion in IP does not replace the warty stage, but accompanies it or the inflammatory one as blebs disappear. In IP, the latter contain eosinophils; there is plasma and cell infiltration ...
متن کاملFundus changes in incontinentia pigmenti ( Bloch - Sulzberger syndrome ) : a case report
The ocular and systemic changes in incontinentia pigmenti are reviewed here and a case is reported with fundus changes which have not been described previously. Incontinentia pigmenti (IP) is characterised by ectodermal defects, with occasional associated mesodermal abnormalities. The typical skin lesions are blue-grey to chocolate-brown arranged along the naevus lines of Blaschko. These occur ...
متن کاملX-linked incontinentia pigmenti or Bloch-Sulzberger syndrome: a case report.
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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ژورنال
عنوان ژورنال: Japanese Journal of Oral Diagnosis / Oral Medicine
سال: 2016
ISSN: 0914-9694,2188-2843
DOI: 10.15214/jsodom.29.135