Lichtenwald D J, Jakubovic H R, Rosenthal D
Department of Medicine, McMaster University, Hamilton, Ontario, Canada.
Arch Dermatol. 1991 Oct;127(10):1545-8.
An unusual adult case of Langerhans cell histiocytosis is presented. The case initially mimicked granuloma annulare, both clinically and histologically. The patient subsequently had development of extensive cutaneous involvement that pathologically revealed a diffuse infiltrate of CD1-positive histiocytic cells containing Langerhans granules. Extensive investigations failed to detect systemic involvement. The patient's cutaneous eruption did not respond to various therapeutic interventions, including phototherapy with oral psoralen with long-wave UV radiation in the A range (PUVA) and systemic vinblastine sulfate. Marked but temporary clinical and histologic improvement was achieved with total body electron beam radiotherapy. The nosology of this case is discussed in the context of the various histiocytic proliferative disorders.
本文报告了一例不寻常的成人朗格汉斯细胞组织细胞增多症病例。该病例在临床和组织学上最初酷似环状肉芽肿。患者随后出现广泛的皮肤受累，病理检查显示CD1阳性组织细胞弥漫浸润，内含朗格汉斯颗粒。广泛检查未发现系统性受累。患者的皮肤皮疹对各种治疗干预均无反应，包括口服补骨脂素联合A波段长波紫外线光疗（PUVA）及全身应用硫酸长春碱。全身电子束放射治疗取得了显著但短暂的临床和组织学改善。结合各种组织细胞增殖性疾病对该病例的分类学进行了讨论。
