McKenzie Shanice, Vecerek Natalia, Kang Yuna, Knowles Byron, Hogeling Marcia
Division of Dermatopathology, Department of Pathology, David Geffen School of Medicine, University of California, Los Angeles, CA.
Dermatol Online J. 2019 Aug 15;25(8):13030/qt6hr0w31v.
Langerhans cell histiocytosis (LCH) is a rare, clinically heterogeneous disease that most commonly occurs in pediatric populations. Congenital self-limited LCH is a benign variant of LCH. It most commonly presents as a diffuse eruption and reports of single lesion cases are infrequent in the literature. Even in the case of congenital self-limited LCH, there is potential for future multisystem relapse, making long-term follow-up important. We present a case of single lesion self-limited LCH in a full-term male infant with interesting morphology. Physical examination revealed a painless, 6 millimeter, well-demarcated, papule encircled by erythema with central hemorrhage. An infectious workup was negative and a punch biopsy was obtained, which showed a dermal infiltrate of histiocytes consistent with a diagnosis of LCH. The lesion healed without intervention within three weeks. Our case highlights the need for dermatologists to consider LCH in the differential diagnosis for lesions of varying morphology in children, as proper identification is necessary to monitor for multisystem recurrence.
朗格汉斯细胞组织细胞增多症（LCH）是一种罕见的临床异质性疾病，最常见于儿童群体。先天性自限性LCH是LCH的一种良性变体。它最常表现为弥漫性皮疹，而文献中关于单发性病变病例的报道很少。即使是先天性自限性LCH，也有未来多系统复发的可能性，因此长期随访很重要。我们报告一例足月男婴单发性病变自限性LCH，其形态有趣。体格检查发现一个无痛的、6毫米、边界清晰的丘疹，周围有红斑并伴有中央出血。感染性检查结果为阴性，遂进行了打孔活检，结果显示组织细胞浸润于真皮，符合LCH的诊断。该病变在三周内未经干预即愈合。我们的病例强调皮肤科医生在儿童不同形态病变的鉴别诊断中需要考虑LCH，因为正确识别对于监测多系统复发是必要的。
