Telangiectasia macularis eruptiva perstans associated with a sicca complex

Abstract

We present the case of woman in her 50s who developed numerous red-brown telangiectatic macules on her trunk and extremities, as well as persistent dry eyes and dry mouth. Skin biopsy was consistent with telangiectasia macularis eruptiva perstans (TMEP). Serum tryptase was elevated suggesting systemic involvement. Anti-Ro and La were negative. ANA was positive. Salivary gland biopsy revealed a focus score of 3 and immunostains revealed infiltrates of aberrant CD117 positive mast cells. This case suggests a mechanistic role of mastocytosis in salivary compromise.