Cases reported "Pigmentation Disorders"

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1/50. oral manifestations of idiopathic lenticular mucocutaneous pigmentation (Laugier-Hunziker syndrome): a clinical, histopathological and ultrastructural review of 12 cases.

    OBJECTIVE: To present a clinical, histopathological and ultrastructural study on a group of patients affected by idiopathic mucocutaneous pigmentation (Laugier-Hunziker syndrome: LHS). MATERIALS AND methods: Twelve patients were investigated: clinical examination, laboratory tests, and X-ray studies together with light microscopy and electron microscopy were performed in order to diagnose LHS. RESULTS: All cases showed acquired, benign, macular hyperpigmentation of buccal mucosa lips and nails. Histologically, pigmentations are due to an accumulation of melanin in the basal layer keratinocytes and an increase in the number of melanophages in the submucosa and/or papillary dermis. Ultrastructurally there were increased numbers of normal-appearing melanosomes in keratinocytes of the lower epithelium. No evidence of malignant changes were detected. CONCLUSIONS: The importance of this condition relates to it being included in the differential diagnoses of pigmentary disorders of the oral mucosa with associated nail involvement. It is important to recognize this acquired benign disorder to avoid unnecessary investigations and treatments.
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keywords = mucosa
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2/50. lichen planus with involvement of all twenty nails and the oral mucous membrane.

    A 57-year-old man had had deformities of all ten fingernails for one and a half years before presentation and deformities of all ten toenails for the previous six months. The surfaces of the nails were rough, with excessive longitudinal striations. The bases of the nails were slightly hypertrophic, and the tips were atrophic and itchy. A longitudinal nail biopsy including the nail matrix revealed the typical histology of lichen planus. Reticulated pigmentation, maceration, and erosion on the buccal mucous membrane were also discovered. Histological analysis of the buccal mucous membrane revealed lichen planus intermingled with eosinophils. Immunological blood analysis revealed elevated CD4 T cells and CD4/CD8 ratio. He worked as a tinsmith and had dental metal. The metal series patch test revealed positive reactions to chromate and tin. Treatment with systemic steroids was quite effective in treating the nail lesions.
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ranking = 18.18427935561
keywords = mucous membrane
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3/50. Two autopsy cases of diffuse gastrointestinal polyposis with ectodermal changes. Cronkhite-canada syndrome.

    Two autopsy cases of Cronkhite-canada syndrome were reported. The caused of hypoproteinemia, electrolyte imbalance and ectodermal changes were discussed with reference to previously reported cases. The mechanism of protein loss was probably due to outflow into the intestinal lumen of the mucous substance in the cystically dilated glands, directly and/or indirectly followed by loss of mucosal surface. Electrolyte imbalance probably developed from gastrointestinal loss as well as poor substitution. The ectodermal changes were probably not a subsequent part of the emaciation or hypoproteinemis, but an inherent part of this disease. Therapy, whether substitution or surgical procedure, should be selected in order to control the general condition of the patient.
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keywords = mucosa
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4/50. Dyschromatosis universalis hereditaria.

    Dyschromatosis universalis hereditaria is a clinically heterogenous disorder. We report two unrelated Indian patients with dyschromatosis universalis hereditaria, who had generalized and progressive reticulate hyper- and hypo-pigmentation of the skin. The oral mucosa and tongue also showed mottled pigmentation. Intriguingly, the palms and soles were also affected with a diffuse hyper-pigmentation interspersed with spotty de-pigmented macules. Dystrophic nail changes with pterygium formation were seen in one case. Histopathology revealed a variable degree of pigmentary incontinence. Although the precise aetiology of this disorder is not yet known, the clinicopathological findings implicate an inherent abnormality of melanosomes or melanin processing.
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ranking = 0.33333333333333
keywords = mucosa
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5/50. Black esophagus induced by severe vomiting in a healthy young man.

    Black esophagus is an uncommon entity that has been described only a few times previously. It is defined as a dark pigmentation of the esophagus associated with histologic mucosal necrosis. Most cases have no known etiology, although ischemia, nasogastric tube trauma, infection, gastric outlet obstruction, gastric volvulus, and hypersensitivity to antibiotics have all been suggested as possible causes. Herein we report the case of a young, healthy, athletic man who developed black esophagus due to severe vomiting after alcohol overindulgence and summarize the other published cases to date.
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keywords = mucosa
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6/50. dyskeratosis congenita associated with three malignancies.

    dyskeratosis congenita is a rare inheritable disorder characterized by abnormalities of the skin, nails and oral mucosa. Aplastic anaemia resulting from bone marrow hypoplasia is a frequent cause of death. Squamous cell carcinoma developing from leukoplakia and visceral malignancies are other complications of the disease. We report here a case of dyskeratosis congenita in a man who developed three neoplasias of different systems over a period of many years. Squamous cell carcinoma and gastric adenocarcinoma manifested 17 years after the man was diagnosed with Hodgkin's disease.
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keywords = mucosa
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7/50. Accelerated phase at initial presentation: an uncommon occurrence in chediak-higashi syndrome.

    The authors describe an Indian child, who presented in the accelerated phase of the chediak-higashi syndrome. The disease usually presents in early childhood with recurrent skin and mucosal infections. This patient had subtle pigmentary abnormalities and no family history of the disease, which made the clinical diagnosis difficult. The cytopenias, hepatosplenomegaly, lymphohistiocytic infiltrate in the bone marrow, and the characteristic granules in the leucocytes clinched the diagnosis. This case underscores the importance of a bone marrow examination in patients with unusual presentations of rare disorders.
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ranking = 0.33333333333333
keywords = mucosa
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8/50. Acral hyperpigmented macules and longitudinal melanonychia in AIDS patients.

    Two patients with AIDS are described who developed acral hyperpigmented macules of the fingers, palms and soles, buccal mucosa and genitalia, associated with longitudinal melanonychia. These pigmentary changes seemed to be independent of zidovudine and were associated in one with diffuse melanoderma and elevated levels of alpha-msh. Histological and ultrastructural studies showed an increase of the dendrites and pigmentation of the melanocytes and few melanosomes in the keratinocytes.
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ranking = 0.33333333333333
keywords = mucosa
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9/50. dyskeratosis congenita: delay in diagnosis and successful treatment of pancytopenia by bone marrow transplantation.

    dyskeratosis congenita is an inherited disorder characterized by nail dystrophy, skin pigmentary changes, mucosal leukoplakia, pancytopenia and an increased incidence of malignancy. Because of a widely held view that the outcome of bone marrow transplantation in dyskeratosis congenita is poor, this treatment option is sometimes not considered when pancytopenia develops. We present a child currently doing well 3 years after bone marrow transplantation, and review the literature.
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ranking = 0.33333333333333
keywords = mucosa
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10/50. Black pigmentation of bone due to long-term minocycline use.

    minocycline-induced dark pigmentation has been described affecting the oral cavity (teeth, mucosa, alveolar bone), skin, nails, eyes and thyroid. To date, there is no report of other bones being affected. We report a case of black pigmentation of the acromian in a patient who had used minocycline on a long-term basis for acne rosecea. biopsy of the iliac crest revealed that the pelvis was also affected.
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ranking = 0.33333333333333
keywords = mucosa
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