• Childhood Kidney Diseases
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• v.10 no.1
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• pp.40-44
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• 2006

[ $Henoch-Sch{\"{o}}nlein$ ] purpura(HSP) is a systemic small vessel vasculitis characterized by cutaneous purpura, arthritis, abdominal pain and nephritis. The characteristic rash of HSP consists of palpable purpura on the buttocks and lower extremities. Bullous lesions often appear in adults with HSP, whereas they are very rare in children with HSP. In this case report, the patient presented with arthralgia and abdominal pain and had hemorrhagic bullae as a prominent manifestation of the disease. The skin biopsy of the patient revealed typical leukocytoclastic vasculitis of dermal vessels and prominent IgA and fibrinogen deposits on capillary walls by direct immunofluorescence. We confirmed the diagnosis of HSP and observed improvement of clinical symptoms and signs within a few days after corticosteroid treatment. We therefore report a case with a review of the literature.

• Archives of Plastic Surgery
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• v.39 no.1
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• pp.59-62
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• 2012

Sweet's syndrome is characterized by clinical symptoms, physical features, and pathologic findings which include fever, neutrophilia, tender erythematous skin lesions, and a diffuse infiltrate of mature neutrophils. This is a report of our experience of Sweet's syndrome with parotitis. A 57-year-old man initially presented with tender swelling on the right cheek similar to parotitis. His symptoms relapsed despite the use of an oral antibiotic agent for 3 weeks. He additionally presented with erythematous papules and plaques on the periocular area and dorsum of both hands. Histiopathologic findings on punch biopsy of the right dorsum of the hand showed superficial perivenular histiocytic infiltration without vasculitis. We confirmed this as histiocytoid Sweet's syndrome and used systemic corticosteroid. After initiation of treatment with systemic corticosteroids, there was a prompt recovery from both the dermatosis-releated symptoms and skin lesions. Sweet's syndrome should be considered in patients with therapy-refractory parotitis and unclear infiltrated nodules. We present a confusing case who initially appeared to have parotitis but turned out to have histiocytoid Sweet's syndrome.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.4 no.2
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• pp.233-237
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• 2001

Kawasaki Disease, an acute systemic vasculitis of unknown etiology, is the leading cause of acquired heart disease in children in many parts of the world. It predominantly affects children under 5 years of age and has many clinical symptoms. We experienced a case of gas forming enterocolitis associated with Kawasaki Disease. Aeromonas hydrophilia was isolated from her stool culture. So, we report the case with a brief review of its literature.

• Clinical and Experimental Pediatrics
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• v.51 no.5
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• pp.462-467
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• 2008

Kawasaki disease is a systemic vasculitis of unknown etiology, usually occurring in infants and young children. Although the etiology of Kawasaki disease remains uncertain, its serious complicationssuch as giant aneurysm formation, coronary arterial stenotic lesions, and thrombotic occlusionhave been proven to cause myocardial ischemia or infarction in patients with Kawasaki disease. To prevent and treat these complications, several modes of therapyincluding long-term anticoagulation, interventional catheterization, and surgical treatmenthave been gradually developed. In this article, we review the cardiovascular complications following Kawasaki disease and the management thereof, which includes thrombolytic therapy, catheter intervention, and coronary artery bypass graft.

• Clinical and Experimental Pediatrics
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• v.58 no.3
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• pp.84-88
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• 2015

Kawasaki disease (KD) is an acute systemic vasculitis that predominantly affects children, and can result in coronary artery lesions (CAL). A patient with KD who is resistant to treatment with intravenous immunoglobulin (IVIG) has a higher risk of developing CAL. Incomplete KD has increased in prevalence in recent years, and is another risk factor for the development of CAL. Although the pathogenesis of KD remains unclear, there has been increasing evidence for the role of genetic susceptibility to the disease since it was discovered in 1967. We retrospectively reviewed previous genetic research for known susceptibility genes in the pathogenesis of KD, IVIG resistance, and the development of CAL. This review revealed numerous potential susceptibility genes including genetic polymorphisms of ITPKC, CASP3, the transforming growth factor-${\beta}$ signaling pathway, B lymphoid tyrosine kinase, FCGR2A, KCNN2, and other genes, an imbalance of Th17/Treg, and a range of suggested future treatment options. The results of genetic research may improve our understanding of the pathogenesis of KD, and aid in the discovery of new treatment modalities for high-risk patients with KD.

• Childhood Kidney Diseases
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• v.1 no.2
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• pp.176-178
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• 1997

Henoch-Scholein purpura is a systemic vasculitis of small blood vessels. It is characterized by nonthrombocytopenic purpura, abdominal pain, arthralgia and renal involvement. In Henoch-Scholein purpura, severe abdominal pain may be relived by steroid, but occasionally unresponsive to steroid and conventional analgesics therapy. We tried IV-gamma globulin for severe abdominal pain, unresponsive to steroid, analgesics, and antispasmodics therapy in HSP, and experienced dramatic symptomatic improvement. So we report three cases of IVIG therapy against severe abdominal pain in HSP with a brief review of literatures

• The Journal of Korean Medicine Ophthalmology and Otolaryngology and Dermatology
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• v.18 no.3
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• pp.127-134
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• 2005

The Henoch-Schnlein Purpura (HSP) is a systemic small vasculitis, characterized by palpable purpura, abdominal pain, hematuria or proteinuria, and arthritis. The etiology is unknown but it is manifested by allergic reaction, so it is called Anaphylactoid Purpura. The prognosis of disease is usually good, but about 50% of patients have experienced recurrences. Oral corticosteroid is treatment of choice, but it cannot prevent recurrence of Disease. We experienced two case of young patients who suffered from the Henoch-Schnlein purpura. We prescripted Mokyangbinrang-hwan, Insamyang-witang, Gwibi-tang. For both two patient, symptoms were much relieved.

• Journal of Medicine and Life Science
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• v.18 no.2
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• pp.35-39
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• 2021

Exaggerated acute and late toxicities following radiotherapy have been reported in patients with pre-existing connective tissue diseases, such as systemic lupus and scleroderma. Behcet's disease (BD) is a relapsing multisystem connective tissue disease characterized by vasculitis in the mucocutaneous, ocular, gastrointestinal, respiratory, neurologic, urogenital, articular, and cardiovascular systems. Data concerning the relationship between radiotherapy toxicity and BD are limited in the literature. Here, we report a case of lung cancer treated with radiotherapy (60 Gy) in a patient with BD. No severe radiation-induced toxicity was observed. Radiation-induced toxicity in patients with BD has also been discussed.

• Childhood Kidney Diseases
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• v.16 no.1
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• pp.51-53
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• 2012

Henoch-Sch$\ddot{o}$nlein purpura (HSP) is the most common vasculitis in children and is characterized by cutaneous purpura, arthritis, abdominal pain, and nephritis. Bullous skin lesions are rare in children. We report a case involving a 9-year-old female with HSP who displayed rapidly evolving hemorrhagic bullae from the primary purpuric lesions during systemic corticosteroid therapy. The bullae disappeared within 7 days of systemic corticosteroid therapy. Some scar lesions of the skin developed on acute phase recovered completely after 6 months. Bullae should not be considered as a poor prognostic factor of HSP and its renal outcome. Skin biopsy in HSP children with bullae is not necessary if clinical diagnostic criteria of HSP are met. However, further evaluation of more pediatric HSP with bullae is needed to get the clearer conclusions. We report a 9-year-old female with HSP who showed the rapidly evolving hemorrhagic bullae from primary purpuric lesions during systemic corticosteroid therapy.

• Tuberculosis and Respiratory Diseases
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• v.66 no.5
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• pp.374-379
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• 2009

Churg-Strauss syndrome (CSS) or allergic granulomatous angiitis is a rare syndrome that is characterized by hypereosinophilic systemic necrotizing vasculitis affecting small- to medium-sized arteries and veins. In general, it occurs in individuals with pre-existing allergic asthma. When CSS appears in patients, it has the following characteristics: eosinophilia of more than 10% in peripheral blood, paranasal sinusitis, pulmonary infiltrates, histological proof of vasculitis with extravascular eosinophils, and mononeuritis multiplex or polyneuropathy. Therapeutic trials dedicated to Churg-Strauss syndrome have been limited due to the rarity of this disorder and the difficulty in making a histological diagnosis. Proper treatment of patients with CSS is not widely known. In this case study, we report on our experience with an unusual patient case, characterized by purpura and a perforation of the small intestine after inadequate steroid therapy.