伴有狮面的蕈样肉芽肿累及毛囊一例

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

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-6720%。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。

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,TCRβand TCRδclonotypes.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 interferonα-2a was given additionally,once every two days.The swelling and pruritus of face relieved obviously after 2 weeks treatment.