伴蕈样肉芽肿的毛囊黏蛋白病一例

A ease of follicular mueinosis progressing into mycosis fungoides

患者男，36岁，躯干四肢泛发毛囊性丘疹、斑片及浸润性斑块伴瘙痒15年余，头面部结节性浸润性肿块伴脱发3个月。发病后第13年无明确的与恶性淋巴瘤相关的临床及组织学证据。发病后第15年组织活检见真皮内及毛囊周围单个核细胞团块状浸润或在胶原束间穿插，部分细胞核大深染，有异形性，并见单个核细胞移入毛囊，部分毛囊上皮水肿破坏、甚至形成囊腔结构，其中充满黏蛋白。阿新蓝染色阳性。免疫组化示：CD45RO＋＋，CD20-，CD4＋＋＋＋，CD8-。该患者在诊断为毛囊黏蛋白病15年后发现合并蕈样肉芽肿。

A 36-year-old man presented with pruritic follicular papules, patches and infiltrated plaques on the trunk and limbs for more than 15 years, and infiltrated swelling and nodules accompanied by trichomadesis on the face and scalp for 3 months. In the fifteenth year of his disease, histology of biopsy specimens from the trunk showed clusters of mononuclear cell infiltrates in dermis and perifollicular areas or between collagen bundles. Some cells had atypical, large and deeply stained nuclei, mononuclear cells migrating into the follicles were observed, and part of the follicular epithelium was edematous and destroyed with formation of cavities and deposition of mucin that was positive for Alcian blue staining. Immunohistochemisty examinations revealed that the lesions were positive for CD45RO and CD4, but negative for CD20 and CDS. However, no histopathological signs of malignancy were found within thirteen years after the onset of follicular mucinosis, and until the fifteenth year, the patient developed mycosis fungoides.