Telangiectasia Macularis Eruptiva Perstans
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Telangiectasia macularis eruptiva perstans (TMEP) is a rare form of cutaneous mastocytosis and presents as multiple, small, reddish-brown macules on the skin of the trunk and extremities. In contrast to other forms of cutaneous mastocytosis which are predominantly seen in children, TMEP occurs in young adults, although cases in children have been reported. The patient may report systemic symptoms such as flushing, tachycardia, respiratory difficulty, hypotension, heartburn, and diarrhea due to release of mediators.
The macular lesions have irregular borders and range in size from 2 to 6 mm in size. Telangiectasia is not always present. Unlike other forms of cutaneous mastocytosis, Darier sign (wheal and flare reaction upon rubbing/stroking the lesion) is absent.
The diagnosis requires demonstration of increased mast cells in skin biopsies in appropriate clinical context. The findings may be subtle with only modest increase in mast cell numbers in perivascular distribution. Giemsa or Toluidine Blue stains are helpful in highlighting metachromatic granules. Treatment is only required for patients with systemic symptoms and consists of oral antihistamines.
The macular lesions have irregular borders and range in size from 2 to 6 mm in size. Telangiectasia is not always present. Unlike other forms of cutaneous mastocytosis, Darier sign (wheal and flare reaction upon rubbing/stroking the lesion) is absent.
The diagnosis requires demonstration of increased mast cells in skin biopsies in appropriate clinical context. The findings may be subtle with only modest increase in mast cell numbers in perivascular distribution. Giemsa or Toluidine Blue stains are helpful in highlighting metachromatic granules. Treatment is only required for patients with systemic symptoms and consists of oral antihistamines.