Cases reported "Pigmentation Disorders"

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1/85. incontinentia pigmenti (Bloch-Sulzberger syndrome) associated with acute granulocytic leukemia.

    incontinentia pigmenti, a syndrome of developmental defects, was found to be associated with acute granulocytic leukemia in a 4-month-old black girl. She has been responsive to antileukemic drugs, and remission has been induced without difficulty after each relapse. Although survival time was expected to be very short because of her age, race, type of leukemia, and initial peripheral leukocyte count at time of diagnosis, she is still alive 35 months after diagnosis of leukemia. One may speculate that the prolonged survival time might be related to the unknown genetic defect.
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ranking = 1
keywords = pigmenti
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2/85. incontinentia pigmenti achromians.

    A patient with incontinentia pigmenti archromians, who was born to consanguineous parents, is described. This is the first reported case from iran, to our knowledge. A number of features of this disease belong more properly to classical incontinentia pigmenti.
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ranking = 5.2792315327791
keywords = incontinentia pigmenti, incontinentia, pigmenti
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3/85. incontinentia pigmenti with unusual features.

    A case is presented of incontinentia pigmenti with severe neurological handicaps and unusual features such as single umbilical artery, pigmented lesion at birth, and biochemical changes including elevated alkaline phosphatase and immunoglobulin m.
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ranking = 3.0396157663896
keywords = incontinentia pigmenti, incontinentia, pigmenti
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4/85. incontinentia pigmenti and defective neutrophil chemotaxis.

    A child with incontinentia pigmenti and chronic erythema multiforme had recurrent bacterial infections. Greatly elevated serum IgE was found. In addition, the patient's neutrophils showed essentially no chemotaxis toward staphylococcus aureus, escherichia coli, or pseudomonas aeruginosa in either patient or control serum. Neutrophil phagocytosis and killing function were normal.
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ranking = 3.0396157663896
keywords = incontinentia pigmenti, incontinentia, pigmenti
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5/85. incontinentia pigmenti in a 21-year-old man.

    We report a case of incontinentia pigmenti in a 21-year-old man. The rarity of this disease in men is emphaxized. Lesions in the reported patient have faded but are still easily discernible. Six other cases are reviewed.
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ranking = 3.0396157663896
keywords = incontinentia pigmenti, incontinentia, pigmenti
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6/85. incontinentia pigmenti et achromians.

    A case is reported, apparently the first of its kind in the literature, of a female child in which there were not only pigmented lesions of incontinentia pigmenti but also hypopigmented lesions of incontinentia pigmenti achromians. The title incontinentia pigmenti et achromians is suggested for this combined disorder. The child also had associated lesions in the form of nail dystrophy, delayed dentition and epilepsy.
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ranking = 7.5188472991687
keywords = incontinentia pigmenti, incontinentia, pigmenti
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7/85. Incontinentia pigmentii acromians with seizure disorder: a case report.

    Incontinentia pigmentii acromians (IPA) is a bizarre, irregularly shaped leukoderma of the trunk and extremities, characterized by marble cake-like whorls or streaks of hypopigmentation whose distribution can be unilateral or bilateral. Mode of inheritance is most consistent with an autosomal dominant type and in majority of the cases, one or more anomalies are present involving dermatological, dental and ocular disorders. A case of a female child, aged 2 1/2 yrs, referred from Pediatric neurology department, Medical College, Trivandrum with Incontinentia pigmentii acromians and seizure disorder is reported. Hamartomatous dental cusps and single maxillary central incisors have been associated with Incontinentia pigmentii acromians. A case of Incontinentia pigmentii acromians with hypoplastic teeth and tongue abnormalities is presented.
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ranking = 1.6
keywords = pigmenti
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8/85. Chronic actinic dermatitis with vitiligo-like depigmentation.

    This report describes two patients suffering from severe chronic actinic dermatitis. Unusual widespread vitiligo-like depigmentation occurred during the course of the disease. The progression of these lesions was triggered by the chronic actinic dermatitis. Loss of pigment and complete absence of tyrosinase positive melanocytes were found in depigmented skin of both cases. Immunohistological investigation of the inflammatory infiltrate in case 2 revealed a predominance of CD-8 positive cytotoxic/-suppressor lymphocytes. Analysing the adjacent pigmented epidermis of progressive depigmenting lesions a dense exocytosis of CD-8 T-cells was notable. This distribution suggests cytotoxic destruction of melanocytes as the cause for the vitiligo-like depigmentation.
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ranking = 0.2
keywords = pigmenti
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9/85. incontinentia pigmenti achromians (Ito).

    A patient with incontinentia pigmenti achromians was reported. She displayed other abnormalities such as a saddle nose, malformed auricles, growth retardation, convulsions and abnormal EEG. Histopathologically, a partial decrease in melanin granules in the basal layer and the presence of melanocytes which showed a weak dopa reaction were the main findings. The hypofunction of melanin production was detected by electron microscopy.
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ranking = 3.0396157663896
keywords = incontinentia pigmenti, incontinentia, pigmenti
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10/85. Hemihypertrophy and primary small intestinal lymphangiectasia in incontinentia pigmenti achromians.

    A rare occurrence of primary small intestinal lymphangiectasia in an 8-year-old girl with incontinentia pigmenti achromians is reported. In addition, she had right sided hemihypertrophy. Though intestinal lymphangiectasia is known to have a few syndromic associations, its co-existence in a child with incontinentia pigmenti achromians has not yet been reported in the literature. Hemihypertrophy is also extremely rare in the latter and only very few instances of an association between the two have been documented previously.
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ranking = 13.437694598337
keywords = incontinentia pigmenti, incontinentia, pigmenti
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