Incontinentia pigmenti or Bloch-Sulzberger syndrome: a rare X-linked genodermatosis
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چکیده
منابع مشابه
Incontinentia pigmenti or Bloch-Sulzberger syndrome: a rare X-linked genodermatosis*
Incontinentia pigmenti is a rare X-linked genodermatosis that affects mainly female neonates. The first manifestation occurs in the early neonatal period and progresses through four stages: vesicular, verruciform, hyperpigmented and hypopigmented. Clinical features also manifest themselves through changes in the teeth, eyes, hair, central nervous system, bone structures, skeletal musculature an...
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 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...
متن کاملBloch-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...
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ژورنال
عنوان ژورنال: Anais Brasileiros de Dermatologia
سال: 2014
ISSN: 0365-0596
DOI: 10.1590/abd1806-4841.20143043