Korean J Dermatol.  2008 Oct;46(10):1303-1308.

Telangiectasia Macularis Eruptiva Perstans: Clinical and Histopathological Aspects

Affiliations
  • 1Department of Dermatology, Busan Paik Hospital, College of Medicine, Inje University, Busan, Korea. skindoctor@paran.com
  • 2Department of Pathology, Busan Paik Hospital, College of Medicine, Inje University, Busan, Korea.
  • 3Go Woon Se Sang Kim Yang Che's Skin Clinic, Busan, Korea.

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.

Keyword

Mast cells per vessel unit; Telangiectasia macularies eruptiva perstans

MeSH Terms

Age of Onset
Female
Glycosaminoglycans
Humans
Male
Mast Cells
Mastocytosis, Cutaneous
Medical Records
Retrospective Studies
Skin
Telangiectasis
Upper Extremity
Urticaria Pigmentosa
Glycosaminoglycans
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