Cases reported "Pigmentation Disorders"

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1/4. Alkaptonuric ochronosis presenting as palmoplantar pigmentation.

    We describe a 37-year-old woman who presented with palmoplantar pigmentation, thickening and pitting of 4 years duration. Bluish pigmented patches were seen over the sclera of her eyes. Her lumbar spine showed typical calcification of the intervertebral discs. Addition of Benedict's reagent to a urine sample of the patient gave rise to greenish brown precipitate and brownish black supernatant. Alkalinization of urine turned it black. A biopsy of the palmar lesion demonstrated irregular breaking up, swelling and homogenization of collagen bundles in the reticular dermis. Yellow-brown (ochre coloured) pigment was seen lying within the collagen bundles and also freely in the deeper dermis confirming our clinical diagnosis of alkaptonuric ochronosis. To the best of our knowledge this is probably the second report of alkaptonuria presenting with palmoplantar pigmentation.
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keywords = ochronosis
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2/4. alkaptonuria and ochronosis: case report and review.

    alkaptonuria is a rare genetic disorder in which the enzyme homogentisic acid oxidase is deficient, resulting in the accumulation of homogentisic acid in various bodily tissues. This is a multisystem disorder with a characteristic blue-black discoloration of the skin and cartilage, which is termed ochronosis. Herein we report a profound case of ochronosis secondary to alkaptonuria. Furthermore, we review the clinical manifestations of alkaptonuria and discuss the spectrum of ochronosis, both endogenous and exogenous.
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3/4. Pigmented coalescing papules on the dorsa of the hands: pigmented colloid milium associated with exogenous ochronosis.

    Colloid milium is a rare cutaneous deposition disorder that frequently occurs in areas of chronic sun exposure such as the face, neck and backs of the hands and is characterized by multiple small, discrete, usually amber-colored, dome-shaped papules that cluster to form large plaques. A 50-year-old white woman with esophageal squamous cell carcinoma was referred to us with asymptomatic, slowly spreading lesions localized to the dorsa of her hands which had been present for 4 years. The condition was diagnosed histopathologically as pigmented colloid milium associated with exogenous ochronosis (EO). Colloid milium associated with EO is very rarely reported in the published work. We think that a possible interaction between sunlight and exposure to chemical fertilizers may have played a role in the pathogenesis of both of the disorders in our case.
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keywords = ochronosis
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4/4. Probable coexisting exogenous ochronosis and mercurial pigmentation managed by dermabrasion.

    A patient with blue-gray discoloration of the face is described. Her history revealed that she had used bleaching creams containing mercury and hydroquinone for many years. biopsy specimens of the hyperpigmented areas showed deposits that were compatible with both mercury deposition and the diagnosis of exogenous ochronosis. dermabrasion was successfully employed to remove these deposits.
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keywords = ochronosis
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