No abstract

Hypothenar hammer syndrome (HHS) is an uncommon form of secondary Raynaud phenomenon, occurring mainly in subjects who use the hypothenar part of the hand as a hammer; the hook of the hamate strikes the superficial palmar branch of the ulnar artery in the Guyon space, leading to occlusion and/or aneurysm of the ulnar artery. In patients with HHS, such injuries of the palmar ulnar artery may lead to severe vascular insufficiency in the hand with occlusion of digital artery. To date, only a few series have analyzed the long-term outcome of patients with HHS. This prompted us to conduct the current retrospective study to 1) evaluate the prevalence of HHS in patients with Raynaud phenomenon and 2) assess the short-term and long-term outcome in patients with HHS. From 1990 to 2006, 4148 consecutive patients were referred to the Department of Internal Medicine at the University of Rouen medical center for evaluation of Raynaud phenomenon using nailfold capillaroscopy. HHS was diagnosed in 47 of these 4148 patients (1.13% of cases).Forty-three patients (91.5%) had occupational exposure to repetitive palmar trauma. The more common occupations were factory worker (21.3%), mason (12.8%), carpenter (10.6%), and metal worker (10.6%); the mean duration of occupational exposure to repetitive palmar trauma at HHS diagnosis was 21 years. One patient (2.1%) had recreational exposure (aikido training) to repetitive trauma of the palmar ulnar artery, and 3 other patients (6.4%) developed HHS related to a single direct injury to the hypothenar area. Clinical manifestations were more often unilateral (87.2%) involving the dominant hand (93%). HHS complications included digital ischemic symptoms (ischemia: n = 21, necrosis: n = 20) and irritation of the sensory branch of the ulnar nerve (n = 11). In HHS patients, angiography demonstrated occlusion of the ulnar artery in the area of the Guyon space (59.6%), aneurysm of the ulnar artery in the area of the Guyon space (40.4%), and embolic multiple occlusions of the digital arteries (57.4%). All patients were advised to change their occupational exposure. They were given vasodilators, including calcium channel blocker (n = 37) and buflomedil (n = 12); 36 patients (76.6%) also received oral platelet aggregation inhibitors. Twenty-one patients with digital ischemia/necrosis were further given hemodilution therapy to reduce the hematocrit level to 35%. In 3 patients with HHS-related digital necrosis who exhibited partial improvement with vasodilators, prostacyclin analog therapy (a 5-day regimen of intravenous prostacyclin analog) was instituted, resulting in complete healing of digital ulcer in these 3 patients. Other conservative treatment options included controlling risk factors (smoking cessation, low-lipid diet, therapy for arterial hypertension) and careful local wound care of fingers in the 20 patients with digital necrosis. Only 2 patients, exhibiting digital necrosis and multiple digital artery occlusions, with nonthrombotic ulnar artery aneurysm underwent reconstructive surgery, t...

Our study underlines the high frequency of gastric dysfunction in SSc patients. It suggests the usefulness of EGG in SSc in noninvasively detecting disorders of gastric electrical activity at an early stage and symptomatic patients with gastroparesis (because there was a correlation between values of postprandial to fasting ratio for electrical power of < 1 on EGG and delayed gastric emptying). Gastric-emptying evaluation of radiopaque pellets may further be a helpful method to depict symptomatic SSc patients with gastroparesis. Finally, our series confirms that watermelon stomach diagnosis should be excluded in SSc patients presenting with gastrointestinal hemorrhage or with anemia related to iron deficiency.

Digital ischemia associated with cancer (DIAC) is increasing in frequency and recent reports have suggested the concept of paraneoplastic manifestation. The aims of this study were to characterize the clinical presentation of DIAC and identify clinical features that could lead physicians to diagnose underlying cancer.From January 2004 to December 2011, 100 patients were hospitalized in the Department of Internal Medicine at Rouen University Hospital, France for a first episode of DI. Fifteen (15%) exhibited symptomatic or asymptomatic cancer during the year preceding or following vascular episode and constituted the DIAC group. Other patients without cancer made up the digital ischemia (DI) group.Median time between diagnosis of cancer and episode of digital necrosis was 2 months [0.25–9]. Diagnosis of DI and concomitant cancer was made in 7 of the 15 patients, while DI preceded the malignant disorder in 2 cases and followed it in 6 cases. Histological types were adenocarcinoma for 7 (46.7%), squamous cell carcinoma for 4 (26.7%), and lymphoid neoplasia for 3 patients (20%). Six patients (40%) had extensive cancer. Three patients were lost to follow-up and 5 patients died <1 year after diagnosis of cancer. Cancer treatment improved vascular symptoms in 6 patients (40%). Patients with DIAC, compared to patients with DI, were significantly older (56 years [33–79] vs 46 [17–83] P =0.005), and had significantly lower hemoglobin and hematocrit levels (12.7 g/dl vs 13.9 g/dl; P =0.003 and 38% vs 42%; P =0.003, respectively). Patients with DIAC had a higher platelet rate (420 vs 300 G/L P =0.01), and 6 patients with DIAC (40%) had thrombocytosis. There was no difference between groups either in C-reactive protein level (12 mg/L vs 5 mg/L; P =0.08) or regarding cardiovascular risk factors, presence of autoimmunity, or monoclonal protein.This retrospective study suggests that DIAC may be more prevalent than previously reported. Outcomes of the 2 diseases were not strictly chronologically parallel. However, in the majority of cases, treatment of the tumor resolved vascular involvement. Our findings suggest that age >50 years and thrombocytosis should alert physicians to consider a possible occult malignancy when digital necrosis occurs.