[Malignant melanoma in childhood and adolescence].
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Biomedical subjects
Publications and source records attributed to I Avinoach.
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A rare case of a young Caucasian female with hypopigmented mycosis fungoides is described. We reviewed and discussed the literature.
A classical case of Werner's syndrome is described. In addition to the numerous skin changes that are typically associated with Werner's syndrome, our patients also displayed diffuse lentiginosis, and several of the clinical features of leopard syndrome. Histopathological and ultrastructural findings from a hyperpigmented macule displayed the typical features of a simple lentigo. A striking feature was the presence of melanosomes in Langerhans cells as has been reported in the leopard syndrome. A possible generalized mesodermal defect has been suggested in Werner's syndrome, while the basic defect in the leopard syndrome is thought to be of neuroectodermal origin with pleiotropic changes in the organs derived from the mesoderm. Our patient, with incomplete leopard syndrome and typical Werner's syndrome, may be an example of an association of genetic defects affecting both tissues of neuroectodermal and mesodermal origin.
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A case of gamma/delta-positive T-cell lymphoma localized primarily in the subcutaneous adipose tissue and associated with an extensive hemophagocytic syndrome and a rapid, fatal outcome is presented. Previous reports of gamma/delta cutaneous and subcutaneous T-cell lymphomas are reviewed. To define the characteristics of a T-cell subset of neoplastic cells bearing the gamma/delta receptor in a subcutaneous lymphoma, we undertook clinical and laboratory examinations, morphological evaluation of biopsies, electron microscopy, and extensive immunohistochemical studies. Immunohistochemical studies showed that the neoplastic cells expressed the gamma/delta T-cell receptor (CD3+, C gamma M1+) and were CD2+, CD43+, CD45+, CD45RO+, PCNA+, but were beta F1-, CD1-, CD4-, CD8-, CD15-, CD20-, CD25-, CD30-, CD45R-, CD57-, CD68-, Mac 387-, and HLA Dr-. To the best of our knowledge, this is the first report of a subcutaneous T-cell lymphoma of gamma/delta type involving the subcutaneous tissue and associated with a hemophagocytic syndrome.
BACKGROUND: Spontaneous regression of malignant melanoma is characterized by a partial or complete disappearance of the neoplasm. Partial regression of melanoma has been documented in large series, but complete regression is reported as occurring only sporadically. OBJECTIVE: To describe a case of metastatic malignant melanoma where the primary lesion underwent spontaneous and complete regression and to review the literature pertaining to regression of melanoma. METHODS: The patient was examined clinically. Wide excision of the skin with the lesion was performed as well as left inguinal lymph node dissection. RESULTS: Histologic examination of the lesion revealed complete regression of malignant melanoma. Examination of the lymph node dissected from the adjacent inguinal area revealed matastatic melanoma. CONCLUSIONS: Greater attention should be devoted to identification of regression in melanoma, clinically and histologically, evaluating the prognostic implications which regression might indicate.
We report 2 cases of a pityriasis rosea-like eruption after the use of the anti-inflammatory and antipyretic medications naproxen, acetaminophen, and a combination of acetylsalicylic acid and codeine phosphate. The role of these drugs in the induction of the eruption was supported by the results of macrophage migration inhibition factor and mast cell degranulation tests performed on the offending drugs.
The diagnosis of pemphigus neonatorum in an infant girl was based on clinical, histologic, and immunofluorescence evidence. The outcome was benign, and the skin lesions resolved within two weeks.
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Two unique human melanoma cell lines were established from each of two metastases, with collections separated by a 1-year interval, in a patient with a spontaneously completely regressed primary cutaneous malignant melanoma. These cell lines were distinct, and under culture, they had characteristic features that correlated with those shown by the original tumors from which they were derived. Cells derived from the second metastasis were more aggressive and had a higher proliferative growth rate, serious chromosomal abnormalities, a greater capacity to form colonies on agar, and a lesser dependence on serum-derived growth factors. This study of malignant melanoma cell lines covered the range from the stage of complete spontaneous regression of the primary lesion through the development of the first metastasis (from which the cell line designated L1M1 was established) to the second metastasis (discovered 1 year later, from which the cell line G1M2 was established). These cell lines grow continuously in the laboratory and can be carried for an unlimited number of passages. They afford an opportunity to investigate and compare the malignant pattern and behavior of human malignant melanoma originating after a completely spontaneously regressed primary lesion.
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Hypomelanosis of Ito is an uncommon syndrome where bizarre, systematized hypopigmentation is often associated with neurological and other non-cutaneous abnormalities. We report two cases, one which was associated with laryngomalacia and sudden death (in which the parents were consanguineous), and one which was associated with episodes of loss of consciousness.
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Multiple ulcerated and necrotic lesions developed in a 29-year-old woman. Response to treatment during the next few months was minimal. The most plausible explanation was drug-induced vasculitis caused by contraceptive pills (Microgynon: levonorgestrel 0.15 mg and ethinyl oestradiol 0.03 mg). The diagnosis of vasculitis was confirmed by histopathological and immunofluorescent studies. Urticarial manifestations had developed into necrotizing inflammation and the lesions only began to heal after discontinuation of the pills. Contraceptive pills are not included among the drugs causing necrotizing vasculitis, and to the best of our knowledge, this is the first report. Although contraceptive pills are commonly implicated in the induction of erythema nodusom or vasculitis, such a severe reaction with necrosis is rare.
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Sera of 16 patients with systemic lupus erythematosus (SLE) and active involvement of the CNS were examined for the presence of antibodies to human brain neurons, using indirect immunofluorescence of human brain tissue sections. Thirteen of the 16 patients (81%) had high antineuronal titers, which declined during convalescence, compared with 18 of 105 (17%) SLE patients who had no CNS disease. Competition assays showed that the binding of the antineuronal antibodies was blocked by mycobacterial glycolipids and bovine brain extracts. This finding suggests an additional link between mycobacterial infection and SLE.
The cellular response in the cutaneous leishmaniasis lesion (CL), of BALB/c mice treated topically with an ointment composed of 15% paromomycin and 12% methylbenzethonium chloride (PR-ointment) was studied. In the infected, untreated control group, the lesion showed progressive necrosis with an increase in the number of parasites, macrophages, lymphocytes, and polymorphonuclear cells over a period of 18 weeks. In the PR ointment-treated group, complete healing of the lesion was observed 4 weeks after termination of treatment, but total elimination of the parasites from the lesion was observed only 2 weeks later. A marked reduction in the number of macrophages and polymorphonuclear cells was observed during the healing process. A similar phenomenon was observed with mice inoculated intraperitoneally with paromomycin alone, although total elimination of the parasites from the lesions of these mice was not demonstrated over a period of 18 weeks. Neither L3T4 helper T cells nor Ly2 cytotoxic suppressor T cells were detected in the CL lesion, either before or after treatment.