Abstract：
BACKGROUND: Interstitial mycosis fungoides (IMF) is a rare subtype of mycosis fungoides (MF) characterized by atypical lymphocytes infiltrating the reticular dermis between collagen bundles with limited epidermotropism and variable granulomatous features.
METHODS: Retrospective single institution review of 31 cases of IMF including clinical characteristics, disease course and pathological features.
RESULTS: Our cohort was predominately male (19; 61%, M:F 1.6:1) with a mean age at diagnosis of 43 years (range 11-85), mean signs/symptoms duration of 7 years prior to diagnosis, and 6 years mean follow-up duration. Clinically, patients often exhibited symmetric ill-defined patches/plaques involving intertriginous regions with tan-yellow hyperpigmentation and follicular-based papules, wrinkling, and alopecia. Lymphadenopathy was noted in seven patients. Fifteen (52%) patients were in near or complete clinical remission at the latest follow-up. T-cell receptor gene rearrangement was positive in 23/24 (96%) cases. Histopathologically, atypical cells were small-medium, CD4+ (29; 94%) or rarely CD4+/CD8+ (1; 3%) lymphocytes infiltrating the reticular dermis with thickened collagen bundles (27; 87%), multinucleated giant cells (12; 39%), and often tracing along adnexa with subtle folliculotropism (12/20; 60%).
CONCLUSIONS: Our study demonstrates IMF is an indolent subtype of MF with distinct features, including frequent granulomatous and subtle follicular involvement resulting in alopecia.
METHODS: Retrospective single institution review of 31 cases of IMF including clinical characteristics, disease course and pathological features.
RESULTS: Our cohort was predominately male (19; 61%, M:F 1.6:1) with a mean age at diagnosis of 43 years (range 11-85), mean signs/symptoms duration of 7 years prior to diagnosis, and 6 years mean follow-up duration. Clinically, patients often exhibited symmetric ill-defined patches/plaques involving intertriginous regions with tan-yellow hyperpigmentation and follicular-based papules, wrinkling, and alopecia. Lymphadenopathy was noted in seven patients. Fifteen (52%) patients were in near or complete clinical remission at the latest follow-up. T-cell receptor gene rearrangement was positive in 23/24 (96%) cases. Histopathologically, atypical cells were small-medium, CD4+ (29; 94%) or rarely CD4+/CD8+ (1; 3%) lymphocytes infiltrating the reticular dermis with thickened collagen bundles (27; 87%), multinucleated giant cells (12; 39%), and often tracing along adnexa with subtle folliculotropism (12/20; 60%).
CONCLUSIONS: Our study demonstrates IMF is an indolent subtype of MF with distinct features, including frequent granulomatous and subtle follicular involvement resulting in alopecia.
摘要：
背景：间质真菌病（IMF）是一种罕见的真菌病（MF）亚型，其特征是非典型淋巴细胞浸润胶原蛋白束之间的网状真皮，具有有限的表皮性和可变的肉芽肿特征。
方法：回顾性单机构回顾了31例IMF的临床特征，病程和病理特征。
结果：我们的队列主要是男性（19；61%，M：F1.6：1），诊断时的平均年龄为43岁（范围11-85），诊断前7年的平均体征/症状持续时间，和6年平均随访时间。临床上,患者通常表现出对称性不明确的斑块/斑块，涉及皮肤间区域，伴有黄褐色色素沉着和毛囊性丘疹，起皱,和脱发。7例患者出现淋巴结肿大。在最近的随访中，有15例（52％）患者处于接近或完全缓解的临床状态。23/24（96％）例T细胞受体基因重排呈阳性。组织病理学,非典型细胞是小介质，CD4+(29;94%)或很少有CD4+/CD8+(1;3%)淋巴细胞浸润网状真皮，胶原束增厚(27;87%)，多核巨细胞（12；39%），并且经常沿着附件追踪，具有微妙的毛色性（12/20；60％）。
结论：我们的研究表明，IMF是MF的惰性亚型，具有独特的特征，包括常见的肉芽肿和轻微的卵泡受累，导致脱发。
方法：回顾性单机构回顾了31例IMF的临床特征，病程和病理特征。
结果：我们的队列主要是男性（19；61%，M：F1.6：1），诊断时的平均年龄为43岁（范围11-85），诊断前7年的平均体征/症状持续时间，和6年平均随访时间。临床上,患者通常表现出对称性不明确的斑块/斑块，涉及皮肤间区域，伴有黄褐色色素沉着和毛囊性丘疹，起皱,和脱发。7例患者出现淋巴结肿大。在最近的随访中，有15例（52％）患者处于接近或完全缓解的临床状态。23/24（96％）例T细胞受体基因重排呈阳性。组织病理学,非典型细胞是小介质，CD4+(29;94%)或很少有CD4+/CD8+(1;3%)淋巴细胞浸润网状真皮，胶原束增厚(27;87%)，多核巨细胞（12；39%），并且经常沿着附件追踪，具有微妙的毛色性（12/20；60％）。
结论：我们的研究表明，IMF是MF的惰性亚型，具有独特的特征，包括常见的肉芽肿和轻微的卵泡受累，导致脱发。
