• Thu. Sep 21st, 2023

Sastry PS, Powles RL


Apr 9, 2023

Sastry PS, Powles RL. complications, poor prognosis and require more aggressive treatment1). Corticosteroids are used in patients with recurrent skin lesions, abdominal pain with gastrointestinal bleeding or nephropathy, and cytotoxic agents are used as corticosteroid-sparing agents. Some cases reported a successful resolution of symptoms refractory to corticosteroid and immunosuppressive drugs using plasmapheresis, immunoglobulin or dapsone. Thalidomide is a promising drug for severe, unusual, dermatologic diseases2). Thalidomide has immune modulating effects to reduce cytokine synthesis and to inhibit tumor necrosis factor-alpha synthesis3). In this report, we describe an adult patient who had refractory Henoch-Sch?nlein purpura with severe gastrointestinal bleeding, arthritis and peripheral mononeuropathy. The symptoms were refractory to prednisolone, immunosuppressive drugs and plasmapheresis. We applied thalidomide and symptoms had been improved. CASE Survey A 20-year-old girl was accepted to hospital due to arthralgia on both Mouse monoclonal to FAK ankles and palpable purpura on both hip and legs for 5 times. Physical examination demonstrated multiple purpura on hip and APG-115 legs and buttock and bloating and tenderness on both elbows and ankles (Amount 1). Lab data showed regular blood counts, bloodstream chemistry and urine evaluation. C-reactive proteins was 3.7 ESR and mg/dL was 21 mm/hr. Lab tests for antinuclear antibody, rheumatoid aspect and antineutrophil cytoplasmic antibody (ANCA) had been detrimental. Serum concentrations of immunoglobulin (Ig)G, IgA, IgM, C3 and C4 had been regular. A biopsy specimen of skin damage demonstrated leukocytoclastic vasculitis and immunofluorescent research uncovered IgA depositions on vessels (Amount 2). Open up in another window Amount 1. Palpable purpura over the feet. Open in another window Amount 2. (A) Epidermis biopsy displays necrotizing leukocytoclastic vasculitis (H&E, 200). (B) Immunofluorescent evaluation displays IgA deposition over the wall structure of vessel. She was treated with prednisolone 50 mg/time (1 mg/kg/time) for control of arthralgia which triggered the limited movement of both leg joints. Arthralgia was improved immediately but purpura pass on towards the top encounter and trunk and improved slowly a month later. After six weeks, prednisolone was APG-115 tapered to 20 mg/time. Over the seventh week of hospitalization, she experienced stomach hematochezia and pain as well as the hemoglobin was decreased to 7.0 g/dL. Gastrofibroscopic evaluation revealed hemorrhagic gastritis and APG-115 colonoscopy revealed multiple ulcerations (Amount 3). Biopsy specimens in the colon demonstrated ulcerations with neutrophils infiltration and fibrin thrombi development in vessels (Amount 4). APG-115 Intravenous methyl-prednisolone pulse APG-115 therapy had not been effective for stomach hematochezia and discomfort. She was treated with plasmapheresis for 3 times and cyclophosphamide 750 mg (500 mg/m2), accompanied by prednisolone 30 mg/time. Purpura, arthralgia and hematochezia were much improved and prednisolone was tapered to 15 mg/time then simply. Over the 67th time of hospitalization, she complained of numbness and weakness of both 4th and 5th fingertips, accompanied by a wrist drop. The scholarly study of nerve conduction velocity showed both ulnar nerve mononeuropathy. She was treated with prednisolone 30 mg/time and hydroxychloroquine 200 mg/time and the ulnar nerve mononeuropathy, arthralgia and gastrointestinal bleeding solved but purpura was aggravated. We added 200 mg/time of azathioprine to the individual but it had not been effective. Finally, we utilized 400 mg/time of thalidomide and purpura was improved (Amount 5). After 8 weeks of treatment with thalidomide, purpura disappeared and we tapered thalidomide and stopped then. Open in another window Amount 3. Colonoscopic results present multiple ulcers with hemorrhage. Open up in another window Amount 4. Digestive tract biopsy displays neutrophils and nuclear particles infiltration over the wall structure of vessels (H&E, 400). Open up in another window Amount 5. Improved purpura with pigmentation. Debate Henoch-Sch?nlein purpura is a systemic vasculitis symptoms that affects little vessels (arterioles and venules) and will present a number of clinical manifestations. The etiology of.