Pascual José Carlos, Bañuls José, Albares Maria Pilar, Vergara Gloria, Belinchón Isabel, Silvestre Juan Francisco, Betlloch Isabel
Department of Dermatology, Hospital General de Alicante, Alicante, Spain.
J Cutan Med Surg. 2003 Sep-Oct;7(5):399-402. doi: 10.1007/s10227-002-0144-z. Epub 2003 Sep 24.
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.
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.
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.
肥大细胞增多症是一种罕见疾病，其特征是不同组织中的肥大细胞出现原发性病理性增多。皮肤是最常受累的器官。皮肤肥大细胞增多症，包括色素性荨麻疹、孤立性肥大细胞瘤、弥漫性皮肤肥大细胞增多症和持久性斑疹性血管扩张症（TMEP），通常与系统性肥大细胞增多症相鉴别。TMEP主要以血管扩张性斑疹为特征。
我们报告一例TMEP病例，其临床表现不寻常，表现为孤立的血管扩张性斑疹斑块。在系统性肥大细胞增多症的检查中发现了一例肾透明细胞癌。我们讨论了TMEP的临床和组织学表现及治疗。
TMEP是肥大细胞增多症的一种罕见形式，主要发生于成年人，一般预后良好，很少有发生荨麻疹或出现全身症状的倾向。临床医生在面对进行性非典型血管扩张性病变时应考虑这种疾病。然而，该患者同时发现的恶性肿瘤的意义尚不确定。
