PDF(Pubmed)
Abstract：
Keratoacanthoma (KA) is a fast-growing skin tumor subtype that can be observed as a solitary lesion or rarely as multiple lesions in the context of rare genetic syndromes. Syndromes with multiple keratoacanthoma-like lesions have been documented as multiple self-healing squamous epithelioma (Ferguson-Smith syndrome), eruptive keratoacanthoma of Grzybowski, multiple familial keratoacanthoma of Witten and Zak Muir-Torre syndrome, and incontinentia pigmenti. The treatment approach of those entities is challenging due to the numerous lesions, the lesions\' undefined nature, and the co-existence of other malignant skin tumors. Herein, we report a case of a 40-year-old woman who developed multiple treatment-resistant Ferguson-Smith-like keratoacanthomas with a co-existing large and ulcerated invasive squamous cell carcinoma and microcystic adnexal carcinoma on the scalp. Multiple keratoacanthomas on her extremities were successfully treated with oral acitretin (0.5 mg/kg/day) in combination with topical Fluorouracil (5-FU) 5%, while excision and plastic surgery restoration were performed to treat the ulcerated cancer lesion on her scalp. Due to the interesting nature of this rare syndrome, we performed a literature review including case reports and case series on multiple-KA-like lesions syndromes and focusing on diagnosis and therapy approaches. We also conducted a comparison of patient reports, which included assessing the clinical appearance of the lesions and evaluating the success and progress or the failure of various treatment approaches that were implemented.
摘要：
角化棘皮瘤（KA）是一种快速生长的皮肤肿瘤亚型，在罕见的遗传综合征中可以观察到孤立性病变或很少观察到多发性病变。多发性角化棘皮瘤样病变的综合征已被记录为多发性自愈鳞状上皮瘤（Ferguson-Smith综合征），Grzybowski爆发性角化棘皮瘤，Witten和ZakMuir-Torre综合征的多发性家族性角化棘皮瘤，和色素性不连续症。由于许多病变，这些实体的治疗方法具有挑战性，病变的性质不明确，以及其他恶性皮肤肿瘤的共存。在这里，我们报道了一例40岁女性患者,该患者发展为多重治疗耐药的Ferguson-Smith样角化棘皮瘤,头皮上同时存在大型和溃疡性浸润性鳞状细胞癌和微囊附件癌.用口服阿维酮（0.5mg/kg/天）与局部氟尿嘧啶（5-FU）5％联合成功治疗了四肢多发性角化棘皮瘤，同时进行切除和整形手术修复以治疗头皮上的溃疡性癌症病变。由于这种罕见综合征的有趣性质，我们进行了文献综述,包括多KA样病变综合征的病例报告和病例系列,重点是诊断和治疗方法.我们还对患者报告进行了比较，其中包括评估病变的临床表现，并评估已实施的各种治疗方法的成功和进展或失败。
