BACKGROUND: Mastocytosis is a rare disease characterized by a primary pathological increase in mast cells in different tissues. The skin is the most frequently affected organ. Cutaneous mastocytosis, including urticaria pigmentosa, solitary mastocytoma, diffuse cutaneous mastocytosis, and telangiectasia macularis eruptiva perstans (TMEP), is usually distinguished from systemic mastocytosis. TMEP is characterized mainly by telangiectatic macules. OBJECTIVE AND METHODS: We report a case of TMEP with an unusual clinical presentation as a solitary plaque of telangiectatic macules. A renal clear cell carcinoma was detected in a workup for systemic mastocytosis. We discuss the clinical and histological findings and treatment of TMEP. CONCLUSIONS: TMEP is a rare form of mastocytosis, which occurs mainly in adults, generally has a good prognosis, and little tendency to urticate or show constitutional symptoms. Clinicians should consider this disorder when confronted with a progressive atypical telangiectatic lesion. However, the malignant neoplasm also found in this patient is of uncertain significance.
BACKGROUND:Mastocytosis is a rare disease characterized by a primary pathological increase in mast cells in different tissues. The skin is the most frequently affected organ. Cutaneous mastocytosis, including urticaria pigmentosa, solitary mastocytoma, diffuse cutaneous mastocytosis, and telangiectasia macularis eruptiva perstans (TMEP), is usually distinguished from systemic mastocytosis. TMEP is characterized mainly by telangiectatic macules. OBJECTIVE AND METHODS: We report a case of TMEP with an unusual clinical presentation as a solitary plaque of telangiectatic macules. A renal clear cell carcinoma was detected in a workup for systemic mastocytosis. We discuss the clinical and histological findings and treatment of TMEP. CONCLUSIONS: TMEP is a rare form of mastocytosis, which occurs mainly in adults, generally has a good prognosis, and little tendency to urticate or show constitutional symptoms. Clinicians should consider this disorder when confronted with a progressive atypical telangiectatic lesion. However, the malignant neoplasm also found in this patient is of uncertain significance.