A case of Degos disease successfully treated with corticosteroid combined with cyclophosphamide

Degos disease is a rare disorder characterized by systemic vasculitis involving various organs. There is no established, effective treatment for the disorder, and its prognosis is still poor. Combination therapy with corticosteroid and cyclophosphamide is considered effective for vasculitides involving the small arteries such as ANCA-associated vasculitis. We present here a 42-year-old man who developed Degos disease over several months, and was successfully treated using combined treatment with corticosteroid and cyclophosphamide.     

Effective infliximab treatment for a recurrent type of acute intestinal graft-versus-host disease accompanied by steroid-induced depression

A 22-year-old man with chronic active Epstein-Barr virus infection underwent allogeneic bone marrow transplantation (allo-BMT) from an HLA two allele-mismatched unrelated donor. Ten months after allo-BMT, he developed protein-losing enteropathy following a respiratory syncytial virus infection. A diagnosis of a recurrent type of acute graft-versus-host disease (GVHD) was made based on the histopathological findings, such as the infiltration of T lymphocytes into the superficial epithelium and crypts, and apoptotic bodies in crypts. Although methylprednisolone (mPSL: 10 mg/kg) administration for two consecutive days improved gastrointestinal symptoms, acute pancreatitis and severe depression developed in association with corticosteroid treatment. Reduction of mPSL and administration of infliximab (5 mg/kg/dose, 3 times) resulted in rapid improvement of depression and pancreatitis without aggravating intestinal GVHD. Recent studies have demonstrated that tumor-necrosis-factor (TNF)-α is associated with not only GVHD but also depression and acute pancreatitis. In the present case, anti-TNF-α treatment enabled us to reduce corticosteroid dose without aggravating GVHD, which suggests that this approach might be effective for the treatment of depression and acute pancreatitis.     

Unusual peroneal nerve palsy caused by intraneural ganglion cyst: pathological mechanism and appropriate treatment

The origin of the peroneal intraneural ganglion and the outcome of treatment are still controversial. We report here three cases with peroneal intraneural ganglion and discuss the appropriate treatment. In our cases, 58-, 62-, and 65-year-old patients were operated on with extraneural decompression and epineurotomy within 4 months after onset of drop foot. Two cases demonstrated intraneural ganglion connecting to the articular branch and traversing to the deep and common peroneal nerve. At the 1-year follow-up, paralyzed peroneal nerve could be recovered in all patients even with residual ganglion. We propose correct early diagnosis, simple exoneural dissection, and atraumatic epineurotomy for the successful treatment of peroneal intraneural ganglion. Disruption of the stalk in the articular branch is a key point to prevent recurrence. For early diagnosis, clinicians should be aware of the existence of this rare lesion.