Ludolph-Hauser D, Schöpf P, Rueff F, Przybilla B
Klinik und Poliklinik für Dermatologie und Allergologie, Ludwig-Maximilians-Universität, Frauenlobstrasse 9-11, 80337 München.
Hautarzt. 2001 May;52(5):390-3. doi: 10.1007/s001050051330.
Mastocytosis is diagnosed without difficulty if it presents with easily recognizable lesions of urticaria pigmentosa. Recently, we have identified hardly visible skin lesions of mastocytosis in Hymenoptera venom allergic patients ("occult mastocytosis"). In addition, in approximately 15% of the patients with typical cutaneous lesions, urticaria pigmentosa was at first mistaken for other conditions and thus not linked to simultaneous symptoms of systemic mastocytosis. In most patients with unrecognized mastocytosis, the diagnosis was supported by raised basal serum tryptase levels. Cutaneous mastocytosis is often overlooked and more frequent than assumed. Measurement of basal serum tryptase concentrations can make an important contribution to the diagnosis of mastocytosis, but it does not replace a meticulous skin examination.
如果肥大细胞增多症表现为易于识别的色素性荨麻疹皮损,则不难诊断。最近,我们在膜翅目毒液过敏患者中发现了难以察觉的肥大细胞增多症皮肤损害(“隐匿性肥大细胞增多症”)。此外,在大约15%有典型皮肤损害的患者中,色素性荨麻疹起初被误诊为其他疾病,因此未与系统性肥大细胞增多症的同时症状联系起来。在大多数未被识别的肥大细胞增多症患者中,基础血清类胰蛋白酶水平升高支持了诊断。皮肤肥大细胞增多症常常被忽视,且比预期更为常见。基础血清类胰蛋白酶浓度的测定对肥大细胞增多症的诊断有重要帮助,但它不能替代细致的皮肤检查。