Lee Woo Jin, Yun Sook Jung, Jung Joon Min, Ko Joo Yeon, Kim Kwang Ho, Kim Dong Hyun, Kim Myung Hwa, Kim You Chan, Kim Jung Eun, Na Chan-Ho, Mun Je-Ho, Park Jong Bin, Park Ji-Hye, Park Hai-Jin, Shin Dong Hoon, Shin Jeonghyun, Oh Sang Ho, Yun Seok-Kweon, Lee Dongyoun, Lee Seok-Jong, Lee Seung Ho, Lee Young Bok, Cho Soyun, Choi Sooyeon, Choi Jae Eun, Lee Mi Woo
Department of Dermatology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
Department of Dermatology, Chonnam National University Medical School, Gwangju, Korea.
Ann Dermatol. 2025 Apr;37(2):75-85. doi: 10.5021/ad.24.120.
Primary cutaneous CD30+ lymphoproliferative disorders (pcCD30-LPDs) are a diseases with various clinical and prognostic characteristics.
Increasing our knowledge of the clinical characteristics of pcCD30-LPDs and identifying potential prognostic variables in an Asian population.
Clinicopathological features and survival data of pcCD30-LPD cases obtained from 22 hospitals in South Korea were examined.
A total of 413 cases of pcCD30-LPDs (lymphomatoid papulosis [LYP], n=237; primary cutaneous anaplastic large cell lymphoma [C-ALCL], n=176) were included. Ninety percent of LYP patients and roughly 50% of C-ALCL patients presented with multiple skin lesions. Both LYP and C-ALCL affected the lower limbs most frequently. Multiplicity and advanced T stage of LYP lesions were associated with a chronic course longer than 6 months. Clinical morphology with patch lesions and elevated serum lactate dehydrogenase were significantly associated with LPDs during follow-up in LYP patients. Extracutaneous involvement of C-ALCL occurred in 13.2% of patients. Lesions larger than 5 cm and increased serum lactate dehydrogenase were associated with a poor prognosis in C-ALCL. The survival of patients with C-ALCL was unaffected by the anatomical locations of skin lesions or other pathological factors.
The multiplicity or size of skin lesions was associated with a chronic course of LYP and survival among patients with C-ALCL.
原发性皮肤CD30+淋巴增殖性疾病（pcCD30-LPDs）是一类具有多种临床和预后特征的疾病。
增加我们对pcCD30-LPDs临床特征的认识，并在亚洲人群中确定潜在的预后变量。
对从韩国22家医院获取的pcCD30-LPD病例的临床病理特征和生存数据进行了研究。
共纳入413例pcCD30-LPDs病例（淋巴瘤样丘疹病[LYP]，n = 237；原发性皮肤间变性大细胞淋巴瘤[C-ALCL]，n = 176）。90%的LYP患者和大约50%的C-ALCL患者有多处皮肤病变。LYP和C-ALCL最常累及下肢。LYP病变的多发性和晚期T分期与病程超过6个月相关。在LYP患者随访期间，斑片样病变的临床形态和血清乳酸脱氢酶升高与LPDs显著相关。13.2%的C-ALCL患者出现皮肤外受累。病变大于5 cm和血清乳酸脱氢酶升高与C-ALCL的预后不良相关。C-ALCL患者的生存不受皮肤病变的解剖位置或其他病理因素的影响。
皮肤病变的多发性或大小与LYP的病程及C-ALCL患者的生存相关。
