Telangiectasia Macularis Eruptiva Perstans: Clinical and Histopathological Aspects / 대한피부과학회지

ABSTRACT

BACKGROUND: Telangiectasia macularis eruptiva perstans (TMEP) is a rare form of cutaneous mastocytosis, and this is characterized by multiple brownish-red confluent macules and telangiectasia. Although several cases of TMEP have been reported in the Korean literature, no clinicopathological studies have yet been reported. OBJECTIVE: This study was undertaken to document the clinical features of TMEP, to examine the histopathological features of TMEP and to quantify the number of perivascular mast cell by using the 'mast cells per vessel unit method (MPV). METHODS: The medical records of 16 patients with TMEP and who were seen at our institution from 1999 through 2006 were retrieved. We retrospectively reviewed the medical records, clinical photographs and histopathology slides of these patients. Furthermore, we quantified the number of perivascular mast cells, using the MPV method, on the slides of the TMEP patients, and then we compared this to the number of perivascular mast cells in normal skin (n=10) and urticaria pigmentosa skin (UP, n=10) as controls. RESULTS: The ratio of male to female was 13:3 and the mean age of onset was 36.31+/-12.34 years. The most commonly involved sites were both the trunk and upper extremities (62.5%). 5 patients (31.3%) showed constitutional symptoms. A microscopic examination demonstrated scant perivascular infiltrate with mast cells. The average number of mast cells less than 55micrometer of vessel diameter in the TMEP, normal skin and UP was 3.32, 1.17 and 5.89, respectively and the average number of mast cells that were more than 55micrometer in the TMEP, normal skin and UP was 7.00, 2.08 and 21.00, respectively and these values showed statistical differences (p<.0001). The MPV that was of less than 55micrometer of vessel diameter in the patients with associated cutaneous or systemic symptoms was greater that that of the patients without associated symptoms and these values showed statistical differences (p<.005). CONCLUSION: This study suggested that TMEP can be associated various constitutional and cutaneous symptoms. The MPV method can be an effective method for differentiating the histopathologic findings of TMEP from those of normal skin or UP.