Cases reported "Pigmentation Disorders"

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1/7. prurigo pigmentosa: role of ICAM-1 in the localization of the eruption.

    Immunohistochemical studies were carried out on the skin lesions of two cases of prurigo pigmentosa. There was a predominance of CD4 cells in the dermal infiltrate, whereas those lymphocytes in the epidermis were mainly CD8 cells. The majority of dermal and epidermotropic lymphocytes showed an intense expression of lymphocyte function-associated antigen 1 (LFA-1). The number of CD1 cells was increased in the epidermis. There was intense expression of ICAM-1 by keratinocytes in the erythematous papules. Focal expression of ICAM-1 was still observed in the residual pigmented areas and could explain the recurrence of the lesions at these sites.
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keywords = antigen
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2/7. erythema dyschromicum perstans. Immunopathologic studies.

    Immunopathologic studies of a patient with clinical and histologic evidence of erythema dyschromicum perstans revealed Ia antigen expression on epidermal keratinocytes, pronounced OKT4 and OKT6 staining of epidermal dendritic cells, and dermal infiltration of T lymphocytes of both helper-inducer (OKT4) and suppressor-cytotoxic (OKT8) phenotypes--a pattern commonly associated with lichen planus. These findings, taken in conjunction with positive IgG staining on colloid bodies, suggest the possibility that erythema dyschromicum perstans and lichen planus have similar disease processes.
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keywords = antigen
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3/7. Agenesis of the corpus callosum, combined immunodeficiency, bilateral cataract, and hypopigmentation in two brothers.

    We describe 2 brothers with a malformation syndrome consisting of agenesis of the corpus callosum, cutaneous hypopigmentation, bilateral cataract, cleft lip and palate, and combined immunodeficiency. The clinical history of both patients was characterized by severe psychomotor retardation, seizures, recurrent severe respiratory infections, and chronic mucocutaneous candidiasis. The children died of bronchopneumonia at age 2 and 3 years, respectively. Immunological investigations showed, in one sib studied, skin anergy to recall antigens, profound depletion of T4 lymphocytes, and serum IgG2 deficiency. Necropsy showed agenesis of the corpus callosum, hypoplasia of the cerebellar vermis, and profound hypoplasia of the thymus and of the peripheral lymphoid tissue. The distinctive features of these sibs appear to define a previously undescribed hereditary MCA/MR syndrome. The clinical and pathological findings seem to indicate, as a pathogenetic mechanism, a defect involving the embryonic organization of the central nervous system and of the immune system.
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ranking = 1
keywords = antigen
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4/7. Inflammatory acquired oral hyperpigmentation: association with melanophages demonstrating phenotypic characteristics of antigen presenting cells and activated monocytes.

    A case of a 30-year-old black woman who developed acute, extensive intraoral hyperpigmentation is reported. Its relationship to previously described entities is discussed. The condition was associated with the presence of melanophages both in the submucosa and in the oral epithelium. The melanophages displayed phenotypic markers of a population of intraepidermal melanophages previously described as potent T cell-activating, antigen presenting cells (T6- DR OKM5 OKM1-) and of activated monocytes (Mo3e ), as well as other monocyte-macrophage markers (Mono 1 and My7). The distinct phenotype of these melanophages suggests that they could play an active role in the promotion of inflammatory disease.
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ranking = 5
keywords = antigen
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5/7. Cronkhite-canada syndrome: report of a case with bacteriologic, immunologic, and electron microscopic studies.

    A case of Cronkhite-canada syndrome is presented. The patient developed protein-losing enteropathy, generalized gastrointestional polyposis, skin pigmentation, onychodystrophy, and alopecia. This patient also exhibited certain unusual features such as: fluctuating dermal manifestations, sclerodermalike skin changes, elevated levels of carcinoembryonic antigen, retinal detachment, cataracts, and cerebellar atrophy. Bacteriologic studies of small intestinal fluid, chromosomal analysis, immunologic investigations, and morphologic studies failed to provide any etiologic clues.
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keywords = antigen
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6/7. Schamberg's purpura: association with persistent hepatitis b surface antigenemia and treatment with pentoxifylline.

    A 54-year-old man experienced an extensive asymptomatic purpuric eruption on both his legs consistent with Schamberg's purpura. Three months before, he had had an episode of acute viral hepatitis. Nine months later, the purpura was unchanged despite administration of a topical corticosteroid. Results of serologic evaluation revealed hepatitis b surface antigen. The patient was treated orally with pentoxifylline, 400 mg three times daily. After one month of therapy, the purpuric elements of his eruption had disappeared, and after two additional months most of the pigmentation had also faded.
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ranking = 5
keywords = antigen
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7/7. A single lesion demonstrating features of eccrine poroma and poroid hidradenoma.

    A case of a single poroma lesion with the features of eccrine poroma and poroid hidradenoma is reported. The patient was a 61-year-old woman with a soft nodule, light brown in color, located on the posterior aspect of the right knee and containing a pigmented papular lesion in its center. The histological features of the papular lesion and the nodule were consistent with those of eccrine poroma and poroid hidradenoma, respectively. The eccrine poroma portion consisted mostly of poroid cells. The number of cuticular cells was slightly greater than that of poroid cells in the poroid hidradenoma portion. The cuticular cells in this portion as well as the poroid cells in both portions were immunohistochemically positive for epithelial membrane antigen. The findings in this case suggest that there is a close relationship between eccrine poroma and poroid hidradenoma and support the view that poroid hidradenoma is a type of poroma.
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ranking = 1
keywords = antigen
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