72 patients with angiosarcoma (AS) of the face and scalp have been analyzed with respect of various prognostic factors and the effects of different treatment regimes. This disease predominantly occurs in the elderly (age range, 56-92 years), and affects men rather more frequently than women (men: 44, women: 28). The clinical presentation varied, and included bruise-like lesions, dusky plaques, chronic edema or cellulitis, ulcerated nodules, pyoderma, and infected conditions. Due to lack of clinical awareness and problems with histologic assessment, delays in diagnosis were frequent. The majority of the tumors arose in the upper part of the face or scalp. Less commonly, the central part of the face was affected while only three tumors developed in the mandibular region. Overall the prognosis was poor; one half of the patients died within 15 months of presentation. Only 12% of the patients survived 5 years or more. Patients with lesions that were less than 10 cm in diameter responded better to treatment and statistically survived longer than those with larger lesions, emphasizing the crucial importance of early diagnosis. There is some indication that men, younger patients, patients with lesions on the central part of the face survived longer, but this was not of statistical significance. The histologic differentiation of the tumors at presentation also was of doubtful prognostic significance. Despite the overall poor prognosis, radical radiotherapy (mostly wide-field electron-beam therapy) in seven patients resulted in the apparent eradication of the local skin disease and prolonged survival of the patients. Pulmonary metastases developed 10 years later in two of those patients, however, but the face and scalp remained tumor-free.
A prospective, open, multicentre study was performed to investigate the efficacy and safety of long-term treatment with cyclosporin in adults with severe atopic dermatitis. Subjects were treated for a maximum of 48 weeks. For the first 8 weeks, cyclosporin was administered at 2.5 mg/kg per day. The dose was then adjusted according to response. Disease activity was monitored using the six-area, six-sign score and the proportion of skin involved. Pruritus and sleep disturbance were assessed using four-point scales. Response was further evaluated on a five-point scale. Adverse events, blood pressure and serum biochemistry were monitored. Tolerability was assessed on a five-point scale. One hundred subjects were enrolled and 65 completed 48 weeks of treatment. Withdrawals occurred due to remission (three), inadequate response (seven), protocol violations (11) and adverse events (14, of which seven were probably treatment related). Cyclosporin produced rapid and highly significant improvements in all indices of disease activity. Sixty-five subjects considered that they had shown a considerable improvement or complete clearance of disease. Most patients relapsed after cessation of treatment, but neither signs nor symptoms had returned to baseline severity 8 weeks later. Blood pressure and serum creatinine levels increased slightly, and in one subject renal impairment was a major factor contributing to withdrawal of the drug. Overall, 85 subjects rated the tolerability of cyclosporin as good or very good. The results indicate that cyclosporin has a place in the long-term treatment of severe atopic dermatitis provided that appropriate patients are selected and careful monitoring is performed.
Reactive perforating collagenosis (RPC) is one of the four essential acquired perforating dermatoses. The condition is characterized by the transepidermal elimination of altered collagen. This paper describes four patients with a giant variant of RPC which has not previously been documented. Three of the patients had associated diabetes mellitus and one had chronic renal failure secondary to fetal scarring. Three of the four patients had a significant improvement in their lesions and symptoms following treatment with allopurinol.
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