Gastrointestinal langerhans cell histiocytosis in an adult presenting with anal protrusion and multiple colorectal ulcers: a case report.

Therapeutic advances in rare disease Pub Date : 2024-11-28 eCollection Date: 2024-01-01 DOI:10.1177/26330040241301799
Nhu Tung Tran, Quoc Thanh Truong, Khuyen Thi Nguyen, The Anh Khoa Lai, Cong Thao Trinh, Van Trung Hoang
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Abstract

Langerhans cell histiocytosis (LCH) is a rare disorder characterized by the proliferation of Langerhans cells, a type of dendritic cell essential for immune response. While LCH predominantly affects children, its manifestation in adults, especially within the gastrointestinal (GI) tract, is exceedingly rare. We present a unique case of a 56-year-old female with rare GI manifestations of LCH. The patient initially noticed pimple-like lesions around her anal orifice, which evolved into prominent protruding lesions over 3 months. Subsequent colonoscopy revealed multiple ulcers in the colorectal area, particularly concentrated in the sigmoid colon. Histopathological examination of biopsy samples, combined with immunohistochemical staining, confirmed the diagnosis of LCH. This case underscores the importance of a comprehensive diagnostic approach, especially when patients are present with atypical symptoms. The current literature suggests that such GI manifestations of LCH in adults are infrequent, making this case a valuable contribution to the understanding of LCH's clinical spectrum.

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胃肠朗格汉斯细胞组织细胞增多症在成人表现为肛门突出和多发性结直肠溃疡:1例报告。
朗格汉斯细胞组织细胞增多症(LCH)是一种罕见的疾病,其特征是朗格汉斯细胞(一种对免疫反应至关重要的树突状细胞)的增殖。虽然LCH主要影响儿童,但其在成人中的表现,特别是在胃肠道内,是非常罕见的。我们报告一位56岁女性罕见的LCH胃肠道表现。患者最初发现肛门口周围有丘疹样病变,3个月后演变为突出的病变。随后的结肠镜检查显示结直肠区域多发溃疡,尤其集中在乙状结肠。活检标本的组织病理学检查结合免疫组织化学染色证实了LCH的诊断。该病例强调了综合诊断方法的重要性,特别是当患者出现非典型症状时。目前的文献表明,成人LCH的此类胃肠道表现并不常见,因此本病例对了解LCH的临床谱有重要贡献。
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