Żuber Zbigniew, Dyduch Grzegorz, Jaworek Andrzej, Turowska-Heydel Dorota, Sobczyk Małgorzata, Banach-Górnicka Marta, Rusnak Katarzyna, Górecki Wojciech
Department of Older Children with subunits of Neurology, Rheumatology and Rehabilitation, St. Louis Regional Specialised Children's Hospital, Krakow, Poland.
Department of Pathological Anatomy, Jagiellonian University Medical College, Krakow, Poland.
Reumatologia. 2016;54(3):136-40. doi: 10.5114/reum.2016.61215. Epub 2016 Jul 18.
Pachydermodactyly (PDD) is a rare and benign form of digital soft tissues fibromatosis, which affects the skin of the fingers. The disorder is characterized by asymptomatic, symmetric, progressive soft tissue swelling of the proximal interphalangeal (PIP) joints of the fingers. The etiology of disease remains unknown. It is usually acquired, even though there are some publications that document family cases. It affects mainly adolescent men. We report two boys with the bilateral swelling of the of the PIP joints of the fingers and skin and subcutaneous tissue thickening. Based on clinical manifestations, radiological study and histopathological examination, pachydermodactyly was diagnosed. PDD is a rare and benign disorder, although it is important to consider other diseases, especially rheumatic conditions, in the differential diagnosis in order to avoid unnecessary additional tests and treatments.
厚皮性指端纤维瘤病(PDD)是一种罕见的良性指部软组织纤维瘤病,累及手指皮肤。该病症的特征是手指近端指间(PIP)关节出现无症状、对称、进行性的软组织肿胀。疾病病因尚不清楚。它通常为后天获得性,尽管有一些文献记录了家族病例。它主要影响青少年男性。我们报告了两名手指PIP关节双侧肿胀以及皮肤和皮下组织增厚的男孩。基于临床表现、影像学研究和组织病理学检查,诊断为厚皮性指端纤维瘤病。PDD是一种罕见的良性病症,尽管在鉴别诊断中考虑其他疾病,尤其是风湿性疾病很重要,以便避免不必要的额外检查和治疗。