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类脂质渐进性坏死型蕈样肉芽肿伴无汗,类似瘤型麻风。

Granulomatous mycosis fungoides with hypohidrosis mimicking lepromatous leprosy.

机构信息

Department of Dermatology, Seth GS Medical College and KEM Hospital, Mumbai, India.

出版信息

Indian J Dermatol Venereol Leprol. 2010 Nov-Dec;76(6):686-90. doi: 10.4103/0378-6323.72470.

DOI:10.4103/0378-6323.72470
PMID:21079314
Abstract

Granulomatous mycosis fungoides (GMF) is a rare type of cutaneous T cell lymphoma. A 38-year-old married male presented with decreased sweating all over the body for last 8 years, progressive redness and scaling over body for 2 years and multiple noduloulcerative lesions over the body for 1 year. Cutaneous examination revealed generalized erythema and scaling with poikilodermatous changes over chest and upper back along with multiple noduloulcerative lesions. Skin biopsy from a nodular lesion revealed dense granulomatous infiltrate of atypical lymphocytes with epidermotropism and sparing of appendages. Diagnosis of GMF was made. Computed tomographic scan of thorax, abdomen and pelvis revealed axillary and inguinal lymphadenopathy. Immunohistochemistry revealed leukocyte common antigen and CD3 positivity suggestive of T cell origin. Patient was started on CHOP (Cyclophosphamide, Hydroxydaunorubicin, Oncovin and Prednisolone) regimen of chemotherapy with marked improvement after three cycles of chemotherapy. This case had some clinical resemblance to lepromatous leprosy.

摘要

蕈样肉芽肿(GMF)是一种罕见的皮肤 T 细胞淋巴瘤。一名 38 岁已婚男性,全身出汗减少 8 年,全身红斑和鳞屑进行性加重 2 年,全身多发性结节溃疡病变 1 年。皮肤检查显示胸部和上背部广泛红斑和鳞屑,伴有皮肤异色病样改变,以及多个结节溃疡病变。从一个结节性病变的皮肤活检显示,有密集的肉芽肿性浸润的非典型淋巴细胞,具有亲表皮性,附属物不受累。诊断为 GMF。胸部、腹部和骨盆的计算机断层扫描显示腋窝和腹股沟淋巴结病。免疫组化显示白细胞共同抗原和 CD3 阳性,提示 T 细胞来源。患者开始接受 CHOP(环磷酰胺、柔红霉素、长春新碱和泼尼松)化疗方案,化疗 3 个周期后有明显改善。该病例在临床上与瘤型麻风有些相似。

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