Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, GA.
Department of Pathology and Laboratory Medicine, Hospital of the University of Pennsylvania, Philadelphia, PA; and.
Am J Dermatopathol. 2021 Aug 1;43(8):574-575. doi: 10.1097/DAD.0000000000001868.
S100-negative CD1a-positive cutaneous histiocytosis is an exceedingly rare histiocytosis that is defined histopathologically by a dense dermal infiltrate of ovoid mononuclear cells with grooved nuclei and ample cytoplasm with variable nuclear atypia and mitoses that are immunohistochemically positive for CD1a and negative for S100 and CD207 (langerin). The histogenesis of S100-negative CD1a-positive histiocytosis is unclear, and its precursor cell has yet to be characterized. Although all cases thus far have been described as benign and occasionally self-resolving, the clinical course and outcome of this disease are not fully understood. This case expands the spectrum of disease associated with S100-negative CD1a-positive histiocytosis given its malignant course.
S100 阴性 CD1a 阳性皮肤组织细胞增生症是一种极其罕见的组织细胞增生症，其组织病理学定义为真皮内弥漫性卵圆形单核细胞浸润，细胞核呈沟状，细胞质丰富，具有不同程度的核异型性和有丝分裂，免疫组化 CD1a 阳性，S100 和 CD207（朗格汉斯细胞）阴性。S100 阴性 CD1a 阳性组织细胞增生症的发生机制尚不清楚，其前体细胞尚未被确定。尽管迄今为止所有病例均被描述为良性且偶尔可自行缓解，但该疾病的临床病程和结局尚不完全清楚。鉴于该病例呈恶性病程，因此扩展了 S100 阴性 CD1a 阳性组织细胞增生症相关疾病谱。
