网状红斑黏蛋白病并发蕈样肉芽肿1例

One case report of mycosis fungoides with the clinical presentations of reticular erythematous mucinosis

报告网状红斑黏蛋白病并发蕈样肉芽肿1例。患者女,42岁。因躯干、上肢红斑14年,皮损增多、加重5年就诊。6年前行皮损组织病理学检查示真皮乳头层及网状层有大量黏蛋白样物质沉积,诊断为网状红斑黏蛋白病。入院皮肤科检查:头部、面部、颈部、躯干、四肢见片状轻度浸润性红斑,部分融合成网状,腹部红斑上可见紫红色浸润性斑块。皮损组织病理检查:真皮内淋巴细胞苔藓样浸润,部分细胞异形,少量浸润细胞移入表皮,形成Pautrier样微脓肿。免疫组化染色结果示:LCA(+++),CD45RO(+++)。诊断:蕈样肉芽肿。

A 42-year-old female patient presented with erythemas over the trunk and arms for 14 years and the skin lesions progressed and deteriorated lasting for 5 years.Six year ago ,the first histopathological examination showed deposits of acid mucopolysaccharides in the papillary and reticular dermis with alcian blue staining positive.Then the diagnosis of reticular erythematous mucinosis（REM）was maded at that time.Physical examination revealed slightly infiltrated plaque-like erythema on the face,scalp,neck,trunk and limbs,some of them coalesced in a reticular pattern,The purple-red plaques were also found on the abdomen.The recent biopsy of the plaque showed that atypical lymphocytes were infiltated in a lichenoid pattern in the dermis.The lymphocytes infiltrated into epidermis and formed the Pautrier＇s microbscesses,The immunohistochemistry study found the infiltrated lymphocytes were positive to LCA（＋＋＋）and CD45RO（＋＋＋）.The patient was diagnosed as mycosis fungoides.