Department of Rheumatic and Immunologic Diseases, The Cleveland Clinic, 9500 Euclid Avenue/Desk A50, Cleveland, OH 44195, USA.
Clin Exp Dermatol. 2009 Dec;34(8):e696-8. doi: 10.1111/j.1365-2230.2009.03432.x. Epub 2009 Jul 3.
Elephantiasis nostras verrucosa (ENV) is an unusual skin condition characterized by dermal fibrosis and hyperkeratotic verrucous lesions resulting from chronic nonfilarial lymphoedema. The condition is similar to 'elephantiasis tropica', in which elephantiasis develops secondary to filariasis. Lymphatic obstruction can be primary or due to various causes such as surgery, tumour, radiation, congestive heart failure or obesity. Recurrent attacks of cellulitis lead to further impairment of lymphatic drainage, causing permanent swelling, dermal fibrosis and epidermal thickening. We report a case of a 56-year-old man with systemic sclerosis (SS), who presented with painful lesions on both legs, consistent with ENV. He developed extensive, fungating, papillomatous lesions on the skin of the legs, toes and dorsa of the feet over a period of 3 years. Histology revealed dense dermal fibrosis, oedema of the papillary dermis and extensive pseudo-epitheliomatous changes. To our knowledge, this is the first report of ENV in which SS was considered to be the primary cause for the impairment of lymphatic flow.
疣状肥厚性象皮病（ENV）是一种不常见的皮肤疾病，其特征为真皮纤维化和角化过度的疣状病变，由慢性非血丝虫性淋巴水肿引起。该病与“热带象皮病”相似，后者由血丝虫病引起象皮病。淋巴阻塞可为原发性，也可由手术、肿瘤、放射、充血性心力衰竭或肥胖等各种原因引起。复发性蜂窝织炎发作导致淋巴引流进一步受损，导致永久性肿胀、真皮纤维化和表皮增厚。我们报告了一例 56 岁男性系统性硬化症（SS）患者，其双腿出现疼痛性病变，符合 ENV 的表现。他在 3 年内出现了广泛的、外生性、乳头状病变，累及腿部、脚趾和足背部皮肤。组织学显示致密的真皮纤维化、乳头真皮水肿和广泛的假上皮瘤样改变。据我们所知，这是首例将 SS 视为淋巴流动受损的主要原因的 ENV 报告。
