Cases reported "Pigmentation Disorders"

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11/17. Cutaneous pigmentation secondary to amiodarone therapy.

    amiodarone (Cordarone) is an iodinated cardiac antiarrhythmic drug that causes a slate-gray discoloration of the sun-exposed skin and a yellow-brown stippling of the cornea. Histopathologically, biopsy specimens of aminodarone pigmentation sites disclose yellow-brown refractile granules in the reticular dermis. These granules were characterized by transmission electron microscopy as being concentrically arranged intralysosomal inclusions ("myelinlike" bodies) in dermal endothelial cells and perivascular smooth-muscle cells. Electron probe x-ray analysis of these same inclusions disclosed definite peaks for iodine, evidence for the presence of amiodarone or a metabolite of the drug at these sites. amiodarone, then, concentrates in lysosomes and causes an accumulation of lipids similar to what has been seen with other cationic amphiphilic compounds, such as the glycosphingolipid stored in Fabry's disease. amiodarone must be recognized as a cause of a drug-induced lipid storage disease with cutaneous and corneal manifestations.
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12/17. Retrocorneal pigmentation secondary to iris stromal melanocytic proliferation.

    A 40-year-old black man had two penetrating keratoplasties as a result of complications from alkali burns sustained many years earlier. Two further penetrating keratoplasties were performed for recurrent retrocorneal pigmentation. Both scanning and transmission electron microscopy demonstrated that the retrocorneal pigmented cells were iris melanocytes, based upon their fusiform or dendritic shapes and measurements of their cytoplasmic melanin granules. Repopulation of Descemet's membrane by the melanocytes was produced by a small remnant of atrophic iris. The pigment granules in the iris melanocytes were somewhat larger than those seen in similar cells from Caucasians or Orientals, but had identical measurements to those studied in an iridectomy specimen from another black patient. The retrocorneal iris melanocytes were able to synthesize banded basement membrane material, interrupted laminar basement membrane, and collagen fibrils. The cause of the exfoliation of the iris melanocytes onto the back of the cornea is unknown, but the discovery that iris melanocytes can proliferate in an ongoing fashion and secrete extracellular matrix provides new insight into the physiology of these cells.
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13/17. Scanning and transmission electron microscopic aspects of the nasal acilia syndrome.

    A 3-month-old boy was admitted with failure to thrive and persistent fevers. During a 4 month hospitalization for treatment of suspected sepsis, persistent purulent nasal discharge developed. Biopsies of his nasal mucosa on 3 separate occasions disclosed thinned respiratory epithelium and a complete absence of cilia when examined by electron microscopy (EM). Despite an initial granulocytopenia and a wide range in T-cell numbers, he did not show any evidence of lower respiratory tract infection. A tracheal biopsy process for EM demonstrated normal ciliated epithelium. This patient appears to have an unrecognized syndrome of normal tracheal cilia but absent nasal cilia.
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14/17. incontinentia pigmenti in arizona Indians including transmission from mother to son inconsistent with the half chromatid mutation model.

    incontinentia pigmenti (IP) is an X-linked dominant disease, usually lethal to males. To explain occasional sporadic IP males, the half chromatid mutation model (Gartler & Francke 1975) has been invoked (Lenz 1975). We here report four cases of American Indians with IP. Two girls had sporadic IP. One affected boy's mother had IP. This is the first report of mother-to-son transmission of IP, indicating that a male with an inherited whole chromatid mutation for IP can escape lethality.
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15/17. adult-onset foveomacular pigment epithelial dystrophy.

    Thirty-three pateints had adult-onset foveomacular dystrophy. The macular lesions typically resulted in a mild to moderate decrease in vision and appeared as small, yellow, bilateral, slightly elevated, pigment epithelial lesions with areas of hyperpigmentation. The characteristic finding on fluorescein angiography was a ring of hyperfluorescence surrounding an area of hypofluorescence. The electro-oculograms were either normal or slightly subnormal in most cases. This dystrophy appears to have an autosomal dominant transmission.
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16/17. Osteopathia striata associated with familial dermopathy and white forelock: evidence for postnatal development of osteopathia striata.

    Osteopathia striata and a macular, hyperpigmented dermopathy were found in a Caucasian woman and her two daughters. Sequential radiographs in one daughter showed that the bone lesions were not present during infancy but developed during early childhood. The skin lesions were not those most often associated with osteopathia striata, but appeared tgo be a unique dermatosis, which also included a hypopigmented forelock. A son had neither osseous nor ectodermal lesions. These abnormalities appear to represent a new syndrome, which is inherited with X-linked or autosomal dominant transmission.
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17/17. carney complex: report of a kindred with predominantly cutaneous manifestations.

    We report a four-generation kindred with the complex of myxomas, spotty pigmentation and endocrine overactivity. This kindred demonstrates a relatively limited phenotypic expression with predominance of cutaneous features. male-to-male transmission confirms the autosomal dominant nature of the condition. We propose that pilonidal sinus may be an associated manifestation in this kindred.
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