The Journal of dermatology
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Case Reports
Subcutaneous emphysema with spontaneous pneumomediastinum and pneumothorax in adult dermatomyositis.
We describe a 32-year-old patient with adult dermatomyositis who developed dyspnea and worsening of pre-existing infarcted skin lesions of the fingers. Chest radiographs showed diffuse hazy reticulonodular infiltration in both lungs, subcutaneous emphysema, pneumomediastinum, and pneumothorax. ⋯ Although dermatomyositis and subcutaneous emphysema are all relatively well-known diseases to dermatologists, the occurrence of spontaneous pneumomediastinum and pneumothorax and subsequent subcutaneous emphysema in connective tissue diseases such as dermatomyositis is unfamiliar. We discuss the possible mechanisms of this condition.
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Most diseases exhibit characteristic profiles of cytokine expression, broadly subdivided into Th1, involving primarily cell-mediated responses, of which Interferon-gamma (INF-gamma), Interleukin-2 (IL-2) and Tumor Necrosis Factor-alpha (TNF-alpha) are hallmarks, and Th2 processes, which often involve activation of the humoral arm of the immune system, resulting in elevated levels of IL-4, IL-5 and IL-10. Psoriasis, a disorder characterized by disfiguring skin lesions and elevated levels of activated CD4+ T helper lymphocytes in both peripheral blood and lesional tissue, exhibits a profile of cytokine expression that includes high levels of TNF-alpha and IFN-gamma, with low IL-5 and IL-10, indicating that immunologically, the pathogenesis of the disease is Th1. In this study, we report the results of an investigation of peripheral blood mononuclear cell cytokine expression among Kuwaiti psoriasis patients; we demonstrated two patterns of IFN-gamma production which may suggest differing pathogeneses. ⋯ Ratios of SI (SI IFN-gamma/SL IL-10) for the psoriatic subjects also were significantly greater for the psoriatic subjects than for the controls (p = 0.003). However, within the psoriatic group, eight patients failed to show the expected elevation of IFN-gamma/IL-10 ratio as a result of high unstimulated levels of IFN-gamma production. The divergence of IFN-gamma expression within the psoriatic group may indicate two different modes of T lymphocyte activation contributing to the pathogenesis of psoriasis in this study.
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A great variety of therapies have been attempted for PHN, including pharmacotherapy and physical therapy. However, there has been no decisive treatment, and reports of the clinical efficacy of all available therapies have been rather controversial. Almost all studies conducted so far have looked only at short-term therapeutic efficacy, and only a few investigators have conducted long-term observations or studies on long-term outcome. ⋯ Although 42.6% of patients were still continuing some treatment, 90.9% were found to be able to take care of themselves. Findings obtained were reviewed and discussed from various viewpoints. Our findings showed that iontophoresis therapy is not only effective at the end of the treatment, but its efficacy is maintained over a long period of time, indicating that it is clinically very useful for the treatment of PHN.
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Primary cutaneous amyloidosis is the deposition of amyloid in the skin without involvement of internal organs. It is easily diagnosed when presented in its typical manifestation. Atypical or rare clinical presentations can pose diagnostic difficulties. ⋯ The diagnosis of PCA was made after skin biopsy which showed amyloid deposits in the skin. This condition can easily be confused with other true poikiloderma skin diseases. Histology is important in confirming the diagnosis.
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Review Case Reports
Phacomatosis pigmentovascularis type IIb associated with Sturge-Weber syndrome and pyogenic granuloma.
A case of phacomatosis pigmentovascularis (PPV) in a 6-year-old girl with Sturge-Weber syndrome, pyogenic granuloma, and other complications is described. It is relatively rare that a complete form of Sturge-Weber syndrome was associated with PPV. A review of the literature on PPV, focusing on total number of reported cases and etiological speculations, is presented. ⋯ The average "density" of mast cells (MCs) per mm2 appearing in the central region of the pyogenic granuloma was calculated to be 86.3/mm2 and that in the adjacent nevus flammeus was 37.9/mm2. The "density" of mast cells in pyogenic granuloma separately calculated from ten other cases was 105.5 +/- 28.6/mm2 (mean +/- SD), compared with that in normal skin, 6.85 +/- 4.9/mm2 (n = 20). There was a significant difference between the two, indicating that MCs are closely associated with angiogenesis in pyogenic granuloma.