Churg-Strauss Syndrome ; pathology ; Diagnosis, Differential ; Giant Cell Arteritis ; pathology ; Granulomatosis with Polyangiitis ; pathology ; Humans ; Mucocutaneous Lymph Node Syndrome ; pathology ; Polyarteritis Nodosa ; pathology ; Purpura, Schoenlein-Henoch ; pathology ; Takayasu Arteritis ; pathology ; Thromboangiitis Obliterans ; pathology ; Vasculitis ; classification ; pathology

The antiphospholipid antibody syndrome is an acquired multisystemic disorder characterized by persistent elevated antiphospholipid antibodies and/or hypercoagulation in veins or arteries, or both. The clinical manifestations of the antiphospholipid antibody syodrome are recurrent thrombosis, fetal loss, thrcenbocytopenia, and various cutaneous lesions. Skin lesions are the first sign of this syndrome in 41% of patients and systemic thrombosis develops in 40% of them. Livedo reticularis is the most common cutaneous finding of the antiphosphotipid antibody syndrome. Although vasculitis has not been frequently noted in antiphospholipid antibody syndrome, some vasculitis such as polyarteritis nodosa, giant cell arteritis, and other nonspecific vasculitides have been found in association with antiphospholipid antibody syndrome. We present a male patient with typical manifestations of leukocytoclastic vasculitis with deep vein thrombosis and positive antiphospholipid antibodies. It suggests that a case of antiphospholipid antibody syndorme was accompanied with cutaneous leukocytoclastic vasculitis.
Antibodies, Antiphospholipid* ; Antiphospholipid Syndrome ; Arteries ; Giant Cell Arteritis ; Humans ; Livedo Reticularis ; Male ; Polyarteritis Nodosa ; Skin ; Thrombosis ; Vasculitis* ; Vasculitis, Leukocytoclastic, Cutaneous ; Veins ; Venous Thrombosis

Acute hemorrhagic edema of childhood is an uncommon form of cutaneous leukocytoclastic vasculitis that occurs in children younger than 3 years. This is characterized by tender edema and rosette-shaped purpuric patches that generally resolve without intervention. We describe a 26-month-old boy with acute hemorrhagic edema of childhood, in whom the disease appeared after an acute respiratory illness. Skin lesions presented with acral edema, and rosette-shaped purpuric plaques on the face and limbs. Some authors consider the disease as a purely cutaneous form of Henoch-Schoenlein purpura, but it is more likely that acute hemorrhagic edema of childhood could be regarded as a distinct entity within leukocytoclastic vasculitis.
Child ; Child, Preschool ; Edema* ; Extremities ; Humans ; Male ; Purpura, Schoenlein-Henoch ; Skin ; Vasculitis ; Vasculitis, Leukocytoclastic, Cutaneous

Acute hemorrhagic edema of infancy (AHEI) is an uncommon form of cutaneous leukocytoclastic vasculitis that occurs in infants and children younger than 2 years. AHEI is characterized clinically by marked peripheral edema and fever as well as large palpable purpuric and ecchymotic skin lesions in a target-like pattern, mainly on the face, ears and extremities, similar to the skin findings of Henoch-Schonlein purpura (HSP). The skin lesions heal spontaneously within one to three weeks and internal organs are rarely affected. We report a case of AHEI occurring in a 23-month-old boy who was initially misdiagnosed as HSP, and was later diagnosed according to his clinical symptoms and histochemical characteristics.
Child ; Ear ; Edema* ; Extremities ; Fever ; Humans ; Infant* ; Male ; Purpura ; Purpura, Schoenlein-Henoch* ; Skin ; Vasculitis, Leukocytoclastic, Cutaneous

Rifampin is one of the first line drugs for treating tuberculosis, but it might be associated with serious adverse effects, including renal failure. We report here on a case of a 57-year-old patient who developed Henoch-Shonlein purpura during antituberculosis therapy that included rifampin. The patient converted to negative on the AFB smear for tuberculosis two weeks after the initial administration of antituberculosis medication. After treatment for 60 days, this patient was diagnosed with Henoch-Shonlein purpura by the purpura lesion on the lower legs, the leukocytoclastic vasculitis, the renal impairment and the pathological examination. After stopping rifampin, the skin lesions disappeared in about 10 days and his renal function gradually improved. This case study showed that Henoch-Schonlein purpura can be caused by rifampin during antituberculosis therapy and we recommend that the use of rifampin should be restrained when clinical symptoms of Henoch-Shonlein purpura are observed.
Acute Kidney Injury ; Humans ; Leg ; Middle Aged ; Purpura ; Purpura, Schoenlein-Henoch ; Renal Insufficiency ; Rifampin ; Skin ; Tuberculosis ; Vasculitis ; Vasculitis, Leukocytoclastic, Cutaneous

Henoch-Schoenlein purpura, also known as anaphylactoid purpura, is characterized by palpable purpura, colicky abdominal pain, gastrointestinal hemorrhage, arthralgias, and renal involvement. Histopathologically, the condition represents a vasculitis, and in fact, it may be the most common vasculitis syndrome affecting children. The pathogenesis of Henoch-Schoenlein purpura remains poorly understood, but it is postulated that an unknown antigenic stimulus causes elevation of circulating IgA and that complement activation leads to necrotizing vasculitis. All of its clinical features are attributable to wide spread vasculits. Abdominal pain is the most common gastrointestinal symptom, but intestinal bleeding and intussusception may occur. Mesenteric vasculitis is a rare but potentially serious complication of systemic vasculitis. It is reported in association with rheumatoid arthritis, systemic lupus erythematosus, scleroderma, polyarteritis nodosa, and giant cell arteritis in adult patients. Typical features are diffuse non-specific abdominal pain progressing on occasion to gastrointestinal hemorrhage, perforation, or more rarely infarction. Fortunately intestinal infarction is a rare complications, but if present carries a high chance of mortality, and swift management of the underlying vasculitis is crucial. We describe here an unusual case of a small intestinal infarction associated with Henoch-Schoenlein purpura caused by mesenteric vasculitis.
Abdominal Pain ; Adult ; Arthralgia ; Arthritis, Rheumatoid ; Child ; Complement Activation ; Gastrointestinal Hemorrhage ; Giant Cell Arteritis ; Hemorrhage ; Humans ; Immunoglobulin A ; Infarction* ; Intussusception ; Lupus Erythematosus, Systemic ; Mortality ; Polyarteritis Nodosa ; Purpura ; Purpura, Schoenlein-Henoch* ; Systemic Vasculitis ; Vasculitis

No abstract available.
Vasculitis, Leukocytoclastic, Cutaneous*

PURPOSE: The aim of this study was to investigate the clinical usefulness of upper gastrointestinal (GI) endoscopy in children with Henoch-Schonlein purpura (HSP). METHODS: We retrospectively analyzed the clinical, endoscopic, and histopathologic records of children with HSP who had been admitted to the Department of Pediatrics of Gil Hospital and underwent upper GI endoscopy between January 2002 and June 2009. Patients were classified into the following two groups for statistical analysis: duodenal involvement (+) and duodenal involvement (-). RESULTS: Fifty-one children with HSP underwent upper GI endoscopy; the mean age was 7.2+/-2.9 years. The upper GI endoscopy showed abnormalities of the duodenum in 38 cases (74.5%), 22 of which had duodenal ulcers. Among the biopsy specimens obtained from the duodenum of 37 cases, 13 cases (35.1%) had leukocytoclastic vasculitis, neutrophil debri, and/or extravasation of RBCs. Steroid use was more frequent in the duodenal involvement (+) group (86.8%) than the duodenal involvement (-) group (53.8%; p=0.02). The mean length of hospitalization was 13.9+/-8.43 days in the duodenal involvement (+) group and 8.1+/-4.62 days in the duodenal involvement (-) group (p=0.003). The recurrence rate was significantly higher in the duodenal involvement (-) group than the duodenal involvement (+) group (p=0.027), whereas none of the other study parameters, such as the age of onset, renal involvement, and steroid use, led to significantly higher or lower recurrence rates. CONCLUSION: These results suggest that duodenal involvement can influence the clinical course and prognosis of HSP in children.
Age of Onset ; Biopsy ; Child ; Duodenal Ulcer ; Duodenum ; Endoscopy ; Endoscopy, Gastrointestinal ; Hospitalization ; Humans ; Neutrophils ; Pediatrics ; Prognosis ; Purpura, Schoenlein-Henoch ; Recurrence ; Retrospective Studies ; Vasculitis ; Vasculitis, Leukocytoclastic, Cutaneous

Tumor necrosis factor alpha (TNF-alpha) inhibitors are used widely to treat patients with active rheumatoid arthritis and ankylosing spondylitis (AS). Although various cutaneous reactions can occur as side effects of TNF-alpha inhibitors, systemic vasculitis requiring withdrawal of the agent and immunosuppressive drugs is rare. A 59-year-old male with AS who had been treated with infliximab for 60 months visited us with complaints of palpable purpura on both legs and severe abdominal pain. Abdominal computed tomography showed diffuse wall thickening of the proximal jejunum and ileum and a skin biopsy revealed leukocytoclastic vasculitis. The patient was diagnosed with Henoch-Schonlein purpura (HSP). Infliximab was discontinued and systemic steroid therapy at 0.5 mg/kg resulted in prompt resolution of the HSP. Here, we report the first case of HSP in a patient with AS after infliximab treatment.
Abdominal Pain ; Antibodies, Monoclonal ; Arthritis, Rheumatoid ; Biopsy ; Humans ; Ileum ; Jejunum ; Leg ; Male ; Purpura ; Purpura, Schoenlein-Henoch ; Skin ; Spondylitis, Ankylosing ; Systemic Vasculitis ; Tumor Necrosis Factor-alpha ; Vasculitis ; Vasculitis, Leukocytoclastic, Cutaneous

Most cases of cytomegalovirus (CMV) colitis occur in adults with severe immuno- deficiency. There have been a few reports involving immunocompetent patients. CMV colitis may occur after colonic mucosal injury in immunocompetent patients. Henoch-Schonlein purpura (HSP) is a common systemic vasculitis in childhood. Diagnostic criteria include palpable purpura with at least one other manifestation; abdominal pain, IgA deposition, arthritis or arthralgia, or renal involvement. To best of our knowledge, we describe the first case of CMV colitis in an immunocompetent patient with preceding HSP. A 38-year-old man presented with a 1-day history of abdominal pain, diarrhea and vomiting. Two-years previously, he underwent a right hemicolectomy due to intussusception. Over a 1-month period, palpable purpura was evident on both arms and legs. Colonoscopy revealed multiple, linear geographic ulcerations at an anastomosis opening site with huge ulceration at the small bowel, which was covered with white exudates. Colonoscopic biopsy showed CMV inclusion bodies and skin biopsy revealed leukocytoclastic vasculitis. The patient was successfully treated with gancyclovir and prednisolone.
Abdominal Pain ; Adult ; Arm ; Arthralgia ; Arthritis ; Biopsy ; Colitis ; Colon ; Colonoscopy ; Cytomegalovirus ; Diarrhea ; European Continental Ancestry Group ; Exudates and Transudates ; Ganciclovir ; Humans ; Immunoglobulin A ; Inclusion Bodies ; Intussusception ; Leg ; Purpura ; Purpura, Schoenlein-Henoch ; Skin ; Systemic Vasculitis ; Ulcer ; Vasculitis ; Vasculitis, Leukocytoclastic, Cutaneous ; Vomiting