Intravascular papillary endothelial hyperplasia (IPEH) is a benign vascular lesion which is thought to represent an unusual form of organizing thrombus. A case of IPEH in the kidney of a 7-year-old girl is described. She suffered from intermittent flank pain and gross hematuria for 6 months. On radiological examinations, well-defined hypoechoic lesions were identified in the medullary portion of the left kidney. A well-demarcated, sponge-like mass was noted on gross examination. It was an intravascular mass lined by a fibrous capsule of various thicknesses. It was characterized by papillary fronds lined with benign endothelial cells. This is the first description of a renal IPEH in a child.

Background Sarcoidosis is a multisystemic granulomatous disorder of uncertain etiology, which is rare in Asia. In particular, subcutaneous sarcoidosis is a specific cutaneous lesion of sarcoidosis that is rarely reported. Objectives The aim of this study is to evaluate the clinicopathological features of 18 pateints with specific subcutaneous sarcoidosis and their relationship with systemic feature of the disease. Methods All patients performed skin biopsy and diagnosed with subcutaneous sarcoidosis from 2003 to 2013 were reviewed. Histological finding and clinical characteristics were analyzed. Results Eighteen patients with specific subcutaneous sarcoidosis were observed. Mean age at diagnosis was 51.4 years and female were dominant (17 patients, 94%). The initial lesions of the patients were 12 infiltrated nodulopapule, 3 maculopapular eruption, and 2 scar sarcoidosis. Subcutaneous lesions were most frequently located in the extremities. Four (22.2%) of 18 patients demonstrated only specific cutaneous involvement during follow-up period and the rest of the patients presented systemic involvement which included 14 hilar and mediastinal lymphadenopathy with or wihout lung parenchymal lesion, 3 arthritis, 2 uveitis, or 1 splenic involvement. Serum angiotensin-converting enzyme level in patients with systemic involvement tends to be higher than those without, although there was no statistical significance. In most of the patients, subcutaneous nodules appeared at the beginning of the disease and only 3 cases present skin nodules within 6 months after diagnosis. Twelve (66.5%) patients were treated with oral steroid and 4 (22.2%) patients treated with hydroxychloroquine or colchicine. The nodules remitted spontaneously in less than 1 year in 2 patients. Three patients present relapse course with lung involvement and were treated with oral steroid with azathioprine, which showed partial remission. Conclusions Specific subcutaneous sarcoidosis is a quite uniform clinicopathological entity usually appearing at the beginning of the disease. It may present with nonsevere systemic involvement and showed favorable clinical outcome. Disclosure of Interest : None declared DOI 10.1136/annrheumdis-2014-eular.5278