1/6. parakeratosis variegata: a possible role of environmental hazards?We report 2 cases of parakeratosis variegata (PV) evolving from lesions beginning with characteristics of ashy dermatosis. Both patients presented with a reticulated, poikilodermatous and hyperpigmented eruption with bizarre coalescent lichenoid papules. histology showed lichenoid epidermotropic infiltrates, more pronounced in case No. 1, consistent with early malignancy. The course was chronic: after more than 10 years, systemic symptoms were not present. In patient No. 1, a monoclonal T-cell population was detected 12 years after the onset of the disease. Both patients had close contact with fertilizers and insecticides. In patient No. 2, the lesions spontaneously regressed within 3 years after cessation of exposure. PV may be a prelymphomatous stage of mycosis fungoides or some closely related cutaneous T-cell lymphoma and does not always evolve into overt malignancy. gene rearrangement detection techniques may be helpful in predicting the course of the disease.- - - - - - - - - - ranking = 1keywords = mycosis fungoides, fungoides, mycosis (Clic here for more details about this article) |
2/6. mycosis fungoides.The case of a 46-year-old woman with poikiloderma vasculare atrophicans is discussed. It is a rare clinical form of patch-stage mycosis fungoides characterized by generalized poikiloderma, atrophy, mottled dyspigmentation, and telangiectases.- - - - - - - - - - ranking = 1.7986985369717keywords = mycosis fungoides, fungoides, mycosis (Clic here for more details about this article) |
3/6. Treatment of a case of mycosis fungoides and one of parapsoriasis en plaque with topical PUVA using a monofunctional furocoumarin derivative, 4,6,4'-trimethylangelicin.A case of plaque stage mycosis fungoides and one of parapsoriasis en plaque were treated with topical puva therapy using a monofunctional furocoumarin derivative, 4,6,4'-trimethylangelicin (TMA). Both patients showed complete clearance of eruptions within 16 treatments. The therapeutic effectiveness of TMA was confirmed by the fact that those eruptions exposed to UVA alone, without TMA application, showed slower and less significant improvement. Histologically, dermal infiltrates of mycosis cells and associated epidermotrophism disappeared almost completely in response to TMA PUVA. No side effects or changes in values in laboratory examinations were observed during treatment.- - - - - - - - - - ranking = 5.0746519130195keywords = mycosis fungoides, fungoides, mycosis (Clic here for more details about this article) |
4/6. parapsoriasis with hyper-IgE.A 56-year-old black man had generalized chronic dermatitis for two years before parapsoriasis en plaque (large type) with hyper-IgE (12,000-22,000 ng/ml) was diagnosed. Lymphocyte cultures showed decreased response to mitogens. Direct immunofluorescence of involved skin showed epidermal and dermal mast cells with bound IgE. parapsoriasis with hyper-IgE may represent a form of "hypersensitive immunosurveillance" allowing a patient to reject a clone of malignant cells and retard transformation from a benign state (parapsoriasis) to a malignant state (mycosis fungoides).- - - - - - - - - - ranking = 1keywords = mycosis fungoides, fungoides, mycosis (Clic here for more details about this article) |
5/6. tilorone hydrochloride in the treatment of T cell lymphoproliferative cutaneous disease.tilorone hydrochloride was used to treat eleven patients with T cell cutaneous disease ranging from pre-sezary syndrome to tumor-stage mycosis fungoides. Cutaneous histologic study, Sezary counts, delayed skin tests, patch tests, and quantitative T cell counts were monitored. The study revealed that the response to tilorone depends in part on the type and stage of disease, as well as the characteristics and responsiveness of the individual lymphocyte population. patients with pre-sezary syndrome are most likely to benefit from tilorone. The effect of tilorone on the T cell population is manifested by changes in responses to patch and skin tests, as well as histologic improvement. Intact immune responses and elevated levels of IgE may be important prognostic clinical parameters in these patients. tilorone is ineffective in patients with mycosis fungoides and advanced sezary syndrome. Keratopathy can be a limiting but reversible complication of therapy. The drug may provide an effective therapeutic step in the treatment of early T cell cutaneous disease or an adjunctive therapy to leukapheresis and chemotherapy.- - - - - - - - - - ranking = 2keywords = mycosis fungoides, fungoides, mycosis (Clic here for more details about this article) |
6/6. parapsoriasis in two children: a clinical, immunophenotypic, and immunogenotypic study.parapsoriasis has been described rarely in childhood; a few reports mention this condition as preceding early mycosis fungoides. We report a boy age 15 and a girl age 8 years with different clinical features of parapsoriasis who were followed clinically and histologically for about four years. immunophenotyping of the skin infiltrate and a T cell receptor gene rearrangement analysis were performed on each. The infiltrate was composed mainly of CD4 and CD45 lymphocytes in the first patient and of CD8 and CD45 in the second. No T cell receptor gene rearrangements were found. The paucity of knowledge about the evolution of this entity in childhood and its relationship to mycosis fungoides makes follow-up critical. The importance of immunophenotyping and immunogenotyping is particularly stressed.- - - - - - - - - - ranking = 2keywords = mycosis fungoides, fungoides, mycosis (Clic here for more details about this article) |