Broesby-Olsen Sigurd, Bygum Anette, Gerdes Anne-Marie, Brandrup Flemming
Department of Dermatology, Odense University Hospital, Odense C, Denmark.
Acta Derm Venereol. 2008;88(1):52-6. doi: 10.2340/00015555-0359.
Multiple self-healing squamous epithelioma of Ferguson-Smith (MSSE) is a rare autosomal dominantly inherited disease, almost exclusively reported in patients of Scottish origin, with recurrent, histologically malignant tumours that undergo spontaneous regression. We report clinical observations in a Danish family with 11 affected patients, which appears to be the first Scandinavian family published with the disease. Anamnestic data and data concerning age of onset, localization, number of tumours and treatment modalities were collected from the family and from medical records, and all living patients were examined. Detailed clinical findings in 6 patients are described, together with a summary of clinical data from all 11 affected family members. The mean age of onset in the family was 52.6 years (range 30-81 years). A total of 44 tumours, chiefly located in sun-exposed areas, were recorded and the average number of tumours per patient was 4 (range 1-23). 61.4% of the tumours were excised, 31.8% regressed spontaneously without treatment. MSSE may thus also affect patients of non-Scottish origin. The disease severity can vary greatly among patients and the onset can be at an advanced age. MSSE constitutes an important model for research into tumour biology.
弗格森 - 史密斯多发性自愈性鳞状上皮瘤（MSSE）是一种罕见的常染色体显性遗传病，几乎仅在有苏格兰血统的患者中报道，其肿瘤具有复发性、组织学上为恶性，但可自发消退。我们报告了一个有11名受累患者的丹麦家族的临床观察情况，这似乎是首个发表的患有该疾病的斯堪的纳维亚家族。从该家族和医疗记录中收集了既往病史数据以及关于发病年龄、肿瘤部位、肿瘤数量和治疗方式的数据，并对所有在世患者进行了检查。描述了6例患者的详细临床发现，以及所有11名受累家族成员的临床数据总结。该家族的平均发病年龄为52.6岁（范围30 - 81岁）。共记录了44个肿瘤，主要位于暴露于阳光下的部位，每位患者的肿瘤平均数量为4个（范围1 - 23个）。61.4%的肿瘤被切除，31.8%未经治疗自发消退。因此，MSSE也可能影响非苏格兰血统的患者。该疾病在患者中的严重程度差异很大，且发病年龄可能较晚。MSSE是肿瘤生物学研究的重要模型。
