Rheumatology Department, Hospital de SantaMaria, Centro Hospitalar Lisboa Norte, Lisbon, Portugal.
J Clin Rheumatol. 2012 Aug;18(5):246-8. doi: 10.1097/RHU.0b013e3182614d08.
Pachydermodactyly is a superficial benign fibromatosis of unknown etiology; it is rare, more frequent in adolescent males, and characterized by painless swelling of the proximal interphalangeal joints(PIP) of the hands. Histologic examination of the skin shows epidermal hyperplasia and increased number of dermal fibroblasts and collagen fibers.We report the case of a 16-year-old adolescent boy who presented swelling of the lateral and dorsal regions of all the metacarpophalangeal and PIP joints of the left hand and PIP and metacarpophalangeal joints of the second and fifth fingers of the right hand, with 3 years of evolution and no arthritis or functional impairment. Results of complementary diagnostic examinations were normal, with the exception of hand ultra sound that showed skin thickening, without synovial proliferation or joint effusion. Skin biopsy confirmed pachydermodactyly. The patient under went aesthetic surgery with good outcome, without recurrence.This rare condition should be distinguished from idiopathic juvenile arthritis and other entities such as knuckle pads syndrome.
厚皮症是一种病因不明的浅表良性纤维瘤病，它较为罕见，更多见于青少年男性，其特征是手部近节指间关节（PIP）无痛性肿胀。皮肤组织学检查显示表皮过度增生，真皮成纤维细胞和胶原纤维数量增加。我们报告了 1 例 16 岁青少年男性的病例，他表现为左手所有掌指关节和 PIP 关节的外侧和背侧以及右手第二和第五指的 PIP 和掌指关节肿胀，病程 3 年，无关节炎或功能障碍。除手部超声显示皮肤增厚，无滑膜增生或关节积液外，其他辅助诊断检查结果均正常。皮肤活检证实为厚皮症。患者接受了美容手术，结果良好，无复发。这种罕见疾病应与特发性青少年关节炎和其他实体疾病（如指垫综合征）相鉴别。
