Multicentric reticulohistiocytosis associated with liver carcinoma: report of a case.

We report a unique case of multicentric reticulohistiocytosis (MRH) associated with liver carcinoma. A 61-year-old man presented with a 4-month history of nonpruritic, generalized, ruby-red papules and nodules, accompanied by fever, joint swelling and difficulty in swallowing. Skin histology showed polymorphic histiocyte infiltration with typical 'ground glass' cytoplasm. Further immunohistochemical studies characterized the lesions as positive for leukocyte common antigen, HLA-DR and CD68. The patient had a history of hepatitis B, and systemic examination, including carcinoma index and type-B ultrasonic examination, revealed high levels of AFP and a solid tumor, which was considered malignant, localized on the right lobe of the liver. Treatment of the liver carcinoma resulted in a significant improvement of the skin symptoms. This is the first case study to report an association between MRH and liver carcinoma. A review of the English-language literature reveals the close linkage between MRH and malignancy. All patients with MRH should be evaluated and monitored carefully to determine the underlying neoplasm.

Cutaneous lesions and visceral involvement of tuberous sclerosis.

BACKGROUND: Tuberous sclerosis (TS) is an autosomal dominant disorder with a significant range of clinical expressions. The involvement of vital organs, such as the brain, kidney, heart and lung is the main cause of death in patients with TS. The aim of this study is to summarize the characteristic cutaneous features and common extracutaneous involvement of TS, which are helpful to the early detection of visceral involvement. METHODS: The analyzed clinical data from 78 patients with TS included those from detailed history, physical and dermatological examination, cranial computed tomography (CT) and magnetic resonance imaging (MRI), abdominal ultrasonography, chest roentgenography, hand and foot X-ray and ophthalmologic examination. RESULTS: The skin, brain and kidney were involved frequently in TS patients. Hypomelanotic macules were the most common and earliest cutaneous lesions. Their number was more than 3 in 81.5% of the patients. They were followed by facial angiofibromas and Shangreen's patch in a decreasing frequency. Forehead plaque, facial angiofibromas and Shagreen's patch appeared in patients at mean age of 2.6, 6.0 and 8.1 years respectively. Cranial CT showed a high positive rate in TS patients. CONCLUSIONS: Cutaneous features of TS are helpful in the early diagnosis of the disease. Hypomelanotic macules are especially important for patients with epilepsy or babies whose number of hypomelanotic macules is more than 3. Cranial CT is of great value in the diagnosis of TS. The involvement of visceral organs such as the brain and kidney should be examined in TS patients.

Tempol, one of nitroxides, is a novel ultraviolet-A1 radiation protector for human dermal fibroblasts.

BACKGROUND: Nitroxide has been reported to have antioxidant and some photoprotective properties. Exposure of skin to ultraviolet-A1 (UVA1, 340-400 nm) can lead to formation of reactive oxygen species, reduction in collagen, and increased expression of matrix metalloproteinases (MMPs). OBJECTIVE: The goal of this research was to determine the effects of 4-hydroxy-Tempo (Tempol), one of nitroxides, in the presence of UVA1 on cytotoxicity, superoxide dismutase enzyme (SOD) activity, lipid peroxidation, and expression of collagen I, collagen III and MMP-1, MMP-3 in human dermal fibroblasts in vitro. METHODS: Fibroblasts were irradiated by a single exposure to UVA1 and at the same time incubated with, or without, Tempol and detected twenty-four hours later. SOD activity and lipid peroxidation, as shown by accumulation malonyldialdehyde (MDA), were detected by biochemical assay. Expressions of collagen I, collagen III (protein levels) and MMP-1, MMP-3 (mRNA level) were detected by ELISA and semi-quantitative reverse transcriptase-PCR separately. RESULTS: Cell survival curve after UVA1 irradiation showed dose dependent decrement pattern and Tempol, between 0.03 and 8 mM, increased cell viability in a dose-effect manner when the cells were exposed to 20 J/cm(2) UVA1. Fifteen Joule per centimetre square of UVA1 significantly inhibited SOD activity and collagen I, collagen III protein levels, while increased MDA level and stimulated MMP-1 and MMP-3 mRNA expression. Tempol reversed these effects caused by UVA1 in some degree or completely and in proper concentration, the results were statistically significant compared with irradiated group. CONCLUSIONS: Tempol had photoprotective properties against UVA1 irradiation in vitro. With antioxidant ability, Tempol inhibited extracellular matrix degradation and preserved collagen production in dermis and may be used as an anti-photoaging agent.

Photochemotherapy inhibits angiogenesis and induces apoptosis of endothelial cells in vitro.

BACKGROUND: Photochemotherapy has long been used in the treatment of psoriasis; however, its mechanism has not been completely elucidated. Psoriasis is now regarded as an angiogenesis-related disease. Recent studies indicated that the inhibition of angiogenesis by photochemotherapy could be an underlying mechanism. It was found that photochemotherapy can downregulate the expression of angiogenic factors in keratinocytes. However, the direct effect of photochemotherapy on endothelial cells has not been studied. METHODS: In this study, we determined the effect of photochemotherapy on the proliferation of human microvascular endothelial cells through MTT (3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide) assay and cell cycle analysis. The migration assay and in vitro tube formation assay were used to investigate the migration properties and tube formation ability of human microvascular endothelial cells after psoralen plus UVA (PUVA) treatment. The apoptosis of endothelial cells elicited by photochemotherapy was also analyzed with fluorescence-activated cell sorting analysis (FACS). RESULTS: UVA (0.8-5.0 J/cm(2)) irradiation with the presence of 8-methoxypsoralen (8-MOP) (300 ng/ml) resulted in a dose-dependent reduction in the cell viabilities of endothelial cells. FACS data showed an accumulation of cells in G0/G1 phase of cell cycle and apoptotic features of cell death after UVA irradiation with psoralen. The migration properties and tube formation ability of endothelial cells were dramatically inhibited by photochemotherapy. CONCLUSION: Our results showed that photochemotherapy inhibits angiogenesis and induces apoptosis of human microvascular endothelial cells in vitro, which may be a possible mechanism of photochemotherapy in the treatment of psoriasis.