Sakurai A, Matsumoto K, Ikeo Y, Nishio S I, Kakizawa T, Arakura F, Ishihara Y, Saida T, Hashizume K
Department of Aging Medicine and Geriatrics, Shinshu University, Matsumoto, Japan.
Endocr J. 2000 Oct;47(5):569-73. doi: 10.1507/endocrj.47.569.
The high frequency of cutaneous manifestations in patients with multiple endocrine neoplasia type 1 (MEN 1) has recently been reported. Since prevalence of some cutaneous diseases varies among different ethnic groups, we examined the frequency of facial angiofibromas in Japanese patients with familial MEN 1. Among 27 patients with germline MEN1 gene mutation and one asymptomatic gene carrier, angiofibromas were identified in 43% (12/28) of the subjects. This frequency was significantly lower than that of Caucasian patients, but nonetheless almost equaled those of pituitary tumors and pancreas endocrine tumors. Angiofibromas should be considered as one of major manifestations in MEN 1 regardless of patients' ethnic origin, and clinicians should pay careful attention to the cutaneous lesions in patients with endocrine tumors.
最近有报道称，1型多发性内分泌肿瘤（MEN 1）患者皮肤表现的发生率较高。由于某些皮肤疾病的患病率在不同种族群体中有所不同，我们调查了日本家族性MEN 1患者面部血管纤维瘤的发生率。在27例患有种系MEN1基因突变的患者和1例无症状基因携带者中，43%（12/28）的受试者被发现患有血管纤维瘤。这一发生率显著低于白种人患者，但仍几乎等同于垂体肿瘤和胰腺内分泌肿瘤的发生率。无论患者的种族如何，血管纤维瘤都应被视为MEN 1的主要表现之一，临床医生应密切关注内分泌肿瘤患者的皮肤病变。
