Cases reported "Pigmentation Disorders"

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1/15. minocycline pigmentation of heart valves.

    minocycline, a derivative of tetracycline, is a broad spectrum antibiotic used in the treatment of gram-positive and gram-negative infections. Benitz et al. (1) were the first to report black discoloration of the thyroid gland in rats, dogs, and monkeys given minocycline. Since that time, there have been numerous reports in the literature describing minocycline related black pigmentation of the skin, thyroid gland, and other sites. We report an unusual case of minocycline induced pigmentation of the cardiac valves and coronary vessels. The pigment stained with Fontana-Masson and was reduced with bleaching. The exact nature of the pigment is unclear; however, various theories have been advocated. ochronosis is another cause of black pigmentation of the heart valves; the clinical history should allow distinction between the two.
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2/15. Intractable livedoid vasculopathy successfully treated with hyperbaric oxygen.

    We describe a new method for treating livedoid vasculopathy. The typical presentation of livedoid vasculopathy includes chronic, recurrent painful ulcers, satellite scar-like atrophy and telangiectasia involving the lower extremities. Histologically, these lesions show areas of ulceration and dermal vessel occlusion without frank inflammatory cell infiltration. There is currently no satisfactory therapy available for this disease. Hyperbaric oxygen (HBO) has recently established itself as one of the most effective methods of treating ischaemic wounds, including diabetic ulcers. We used this therapy in two patients whose lesions were resistant to multiple therapeutic modalities. Not only did their ulcers respond rapidly to the HBO therapy, but the disturbing wound pain also resolved at the same time. To our knowledge, this is the first successful trial of HBO therapy in livedoid vasculopathy. We believe this to be a very promising new therapy for livedoid vasculopathy and to be worth further investigation.
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3/15. Blackening of a choroidal hemangioma after photodynamic therapy.

    BACKGROUND: Photodynamic therapy has recently advanced to a first line treatment of symptomatic circumscribed choroidal hemangiomas. HISTORY AND SIGNS: A sixty-year-old male patient was referred with progressive visual loss in his left eye. visual acuity measured 20/80 and fundus examination revealed a prominent red subretinal lesion superior to the fovea with adjacent pigment epithelial irregularities. Thickening of the central retina was seen on OCT imaging. Sonography revealed a dome-shaped lesion with a maximal thickness of 3.3 mm and high internal reflectivity. Combined fundus indocyanine green and fluorescein angiography showed early filling of large vessels within the tumor. THERAPY AND OUTCOME: The hemangioma was treated with verteporphin photodynamic therapy. Two days after treatment, with vision unchanged, fundus examination revealed blackening of the tumor. Two months later vision increased to 20/40 and the tumor area was atrophic and bright red with a slight hyperpigmented rim. Angiography revealed the disappearance of tumor vessels and choroidal hypoperfusion in the area of treatment. CONCLUSIONS: Choroidal hemangiomas may darken initially after photodynamic therapy. This is most probably caused by large coaguli within the voluminous lacuna-like blood-filled vessel system of the hemangioma that are induced by photothrombosis.
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4/15. imipramine hyperpigmentation: a slate-gray discoloration caused by long-term imipramine administration.

    A 48-year-old white woman, skin type III, had a slate-gray discoloration of the face and dorsa of both hands after ingesting imipramine, 150 mg/day for 5 years. Her iris color was also darkened. One year after cessation of the therapy, the discoloration became lighter. Sun-protected skin showed no discoloration. light microscopy revealed an accumulation of doubly refractile golden yellow granules in the papillary dermis, mostly scattered, with some concentration around the blood vessels but not in the endothelial cells. Electron micrographs showed numerous amorphous electron-dense inclusion bodies in histiocytes, phagocytes, fibroblasts, and dermal dendrocytes. melanosomes were phagocytosed in the same cells but in separated locations. imipramine is structurally related to chlorpromazine and can cause slate-gray discoloration. However, the color of the granules deposited in the papillary dermis is golden-yellow and they are not deposited in endothelial cells.
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5/15. early diagnosis of the retinopathy of incontinentia pigmenti: successful treatment by cryotherapy.

    Prospective examination of the ocular fundi was carried out in the newborn female sibling of a girl with incontinentia pigmenti and bilateral retinopathy. At age 3 months the new baby's peripheral retina was found to be avascular in both eyes. At age 2 years progressive vascular proliferation at the watershed area between normal and abnormal retina led to bilateral vitreous haemorrhage. The new vessel tufts promptly regressed when the avascular area in each eye was treated by single-freeze cryotherapy and the blood in the vitreous absorbed. Cicatricial retinopathy was thereby prevented. Prospective retinal examination of newborn female siblings of children with incontinentia pigmenti is advised, with early cryotherapy recommended if progressive neovascularisation is detected.
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6/15. Perilymphatic atrophy of skin. An adverse side effect of intralesional steroid injections.

    A less frequently reported adverse side effect of local steroid injections has been described. Perilymphatic atrophy results in atrophy of the dermal structures and depigmentation along the course of the lymphatic vessels draining the site of injection. The five cases reported here all occurred in dark-skinned patients. Three of the five cases (Cases Three to Five) resolved spontaneously, the longest taking 9 months from the time of injection. The outcome of two of the patients (Cases One and Two) remain unknown since the patients were lost to follow-up. This report did not form any conclusions from the material reported here, but simply sought to make practitioners aware of the occurrence of this adverse side effect.
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7/15. Polyclonal plasma cell proliferation with systemic capillary hemangiomatosis, endocrine disturbance, and peripheral neuropathy.

    An autopsy case of so-called plasma cell dyscrasia (PCD) of a 37-year-old woman was reported. This case showed typical clinical symptoms of PCD, except for no abnormality in serum immunoglobulins. Besides the typical histological findings often observed with PCD, the autopsy revealed an additional finding of systemic angiomatous proliferation of small blood vessels throughout the body, especially in the skin, kidneys, brain, various lymph nodes, and soft tissue. A peculiar glomerular lesion in this case, similar to membranoproliferative glomerulonephritis, was likely to be a result of proliferation of glomerular capillaries. In addition, this case showed histological evidence of retroperitoneal fibrosis and osteosclerosis. These findings were also considered to be due to the vascular abnormality. Although the etiology of the vascular lesions is obscure, it is emphasized that the abnormality is probably an important cause of the clinical symptoms seen in PCD.
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8/15. A syndrome of multiple fundal anomalies in siblings with microcephaly without mental retardation.

    Chorioretinal and retinal anomalies, among others, have been described in association with true autosomal recessive microcephaly. Accompanying hypertrophy of retinal pigment epithelium has been described in association with Gardner's syndrome. We present the cases of three siblings (two boys and one girl) with true autosomal recessive microcephaly without mental retardation and without associated systemic anomalies who showed hypertrophy of the retinal pigment epithelium similar to that described in Gardner's syndrome. In the boys, these spots were located on a background of fine variations in pigment, with scattered depigmented atrophic areas revealing the sclera in the peripheral and midperipheral fundus. Superadded white spots were located in front of the retinal vessels. The younger boy also had discrete patches of chorioretinal atrophy of 0.5 to 1 disc diameter. The girl, in addition to the hyperpigmented spots, had a diffuse area of chorioretinal atrophy. The anomalies described above suggest that congenital hypertrophy of the retinal pigment epithelium is not unique to Gardner's syndrome.
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9/15. Phacomatosis pigmentovascularis: a new syndrome? Report of four cases.

    We examined four patients who had a combination of extensive nevus flammeus, significant oculocutaneous pigmentation, and severe neurologic alterations. All cases were sporadic. The vascular and neurologic alterations were clinically similar to those observed in the sturge-weber syndrome. The capillary vessels are ultrastructurally different in phacomatosis pigmentovascularis from those of a nevus flammeus in the sturge-weber syndrome; however, melanocytes of normal aspect were present in the middle and deep dermis. The characteristic oculocutaneous pigmentation probably represented a noncoincidental association. The term phacomatosis pigmentovascularis seems appropriate for this apparently new neurocutaneous syndrome.
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10/15. Sneddon's syndrome.

    A 24 year old woman presented with generalised livedo reticularis and progressive intellectual decline. A CT scan of her head showed multifocal low density areas, consistent with cerebral infarction. cerebral angiography revealed occlusive disease of major vessels, an arteriovenous malformation, and moya-moya type anastomoses. There was no other associated systemic illness. Sneddon's syndrome was the provisional diagnosis. Her older brother had the same skin condition and was also shown to have occlusive cerebrovascular disease. The pathogenesis and natural history of this uncommon disease are highlighted.
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