中国麻风皮肤病杂志 ›› 2020, Vol. 36 ›› Issue (6): 352-353.doi: 10.12144/zgmfskin202006352

• 病例报告 • 上一篇    下一篇

伴有狮面的蕈样肉芽肿累及毛囊一例

王亚男,晋红中   

  1. 中国医学科学院北京协和医院皮肤科,北京,100730

  • 出版日期:2020-06-15 发布日期:2020-06-04
  • 通讯作者: 晋红中,E-mail: jinhongzhong@263.net

Mycosis fungoides with leonine facie involving hair follicles: a case report

WANG Yanan, JIN Hongzhong   

  1. Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy Medical Science & Peking Union Medical College, Beijing 100730, China
  • Online:2020-06-15 Published:2020-06-04
  • Contact: JIN Hongzhong, E-mail: jinhongzhong@263.net

摘要: 患者,女,67岁。躯干、四肢斑丘疹4年,面部肥厚红斑2年,伴剧烈瘙痒。皮肤组织病理示棘层及毛囊上皮pautrier微脓肿和真皮单一核细胞带状浸润,异形性明显。免疫组化示CD3、CD4强阳性,Ki-67 20%。TCR基因重排示TCRγ、TCRβ、TCRδ均阳性。诊断:蕈样肉芽肿肿瘤期累及毛囊。给予口服美卓乐24 mg/d,2周后加量至32 mg/d,隔日肌肉注射一次300万IU重组人干扰素α-2a,2周后患者面部肿胀及瘙痒明显消退。

关键词: 蕈样肉芽肿, 狮面, 亲毛囊性蕈样肉芽肿

Abstract: A 67-year-old female presented with maculopapules on her trunk and limbs for 4 years, and hypertrophic erythema on her face with pain and intense itch for 2 years. Biopsy showed pautrier's micro-abscess and a band-like dermal lymphoid infiltration. Immunohistochemistry demonstrated that the staining of CD3 and CD4 was strongly positive, and the proliferation index of Ki-67 was 20%. TCR gene rearrangement showed multiple TCRγ, TCRβ and TCRδ clonotypes. The diagnosis of mycosis fungoides involving hair follicles at tumor stage was made. The patient was treated with oral methylprednisolone 24 mg once a day. The dosage of methylprednisolone was increased to 32 mg/daily after 2 weeks, and 300 IU of intramuscular injections of recombinant interferon α-2a was given additionally, once every two days. The swelling and pruritus of face relieved obviously after 2 weeks treatment.

Key words: mycosis fungoides, leonine facies, folliculotropic mycosis fungoides