• 제목/요약/키워드: Dapsone syndrome

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Dapson 증후군 1례 (A case of dapsone syndrome)

  • 원유종;김옥란;유승택;윤영욱;최두영
    • Clinical and Experimental Pediatrics
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    • 제50권5호
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    • pp.493-496
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    • 2007
  • Dapsone은 나병과 여러 수포성 피부 질환의 치료에 널리 사용되고 있다. 일반적인 투여 용량의 dapsone은 부작용이 드물지만, 피부, 신경계, 위장관, 간, 신장과 혈액학적 합병증을 일으킬 수 있다. 이들 부작용 중 하나인 dapsone 증후군은 약물 투여 수주 후에 발생하여, 예측 불가능하게 진행하며 치명적인 경과를 취할 수 있는 중증 약물 과민 반응이다. 저자들은 dapsone 투여 4주 후에 열, 박탈성 피부염, 황달, 용혈성 빈혈, 늑막 삼출 등의 특징적인 임상 소견을 보인 dapsone 증후군 1례를 경험하였기에 보고하는 바이다.

Severe dapsone hypersensitivity syndrome in a child

  • Choi, So Yoon;Hwang, Ho Yeon;Lee, Jung Hyun;Park, Jae Sun;Jang, Min Soo
    • Clinical and Experimental Pediatrics
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    • 제56권6호
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    • pp.260-264
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    • 2013
  • Dapsone (4,4'-diaminodiphenylsulfone, DDS), a potent anti-inflammatory agent, is widely used in the treatment of leprosy and several chronic inflammatory skin diseases. Dapsone therapy rarely results in development of dapsone hypersensitivity syndrome, which is characterized by fever, hepatitis, generalized exfoliative dermatitis, and lymphadenopathy. Here, we describe the case of an 11-year-old Korean boy who initially presented with high fever, a morbilliform skin rash, generalized lymphadenopathy, hepatosplenomegaly, and leukopenia after 6 weeks of dapsone intake. Subsequently, he exhibited cholecystitis, gingivitis, colitis, sepsis, aseptic meningitis, disseminated intravascular coagulation, syndrome of inappropriate antidiuretic hormone secretion, pneumonia, pleural effusions, peritonitis, bronchiectatic changes, exfoliative dermatitis, and acute renal failure. After 2 months of supportive therapy, and prednisolone and antibiotic administration, most of the systemic symptoms resolved, with the exception of exfoliative dermatitis and erythema, which ameliorated over the following 4 months. Agranulocytosis, atypical lymphocytosis, aseptic meningitis, and bronchiectatic changes along with prolonged systemic symptoms with exfoliative dermatitis were the most peculiar features of the present case.

Treatment of refractory IgA vasculitis with dapsone: a systematic review

  • Lee, Keum Hwa;Hong, Sung Hwi;Jun, Jinhae;Jo, Youngheun;Jo, Woogyeong;Choi, Dayeon;Joo, Jeongho;Jung, Guhyun;Ahn, Sunghee;Kronbichler, Andreas;Eisenhut, Michael;Shin, Jae Il
    • Clinical and Experimental Pediatrics
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    • 제63권5호
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    • pp.158-163
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    • 2020
  • IgA vasculitis, formerly known as Henoch-Schönlein purpura, is a systemic IgA-mediated vasculitis of the small vessels commonly seen in children. The natural history of IgA vasculitis is generally self-limiting; however, one-third of patients experience symptom recurrence and a refractory course. This systematic review examined the use of dapsone in refractory IgA vasculitis cases. A literature search of PubMed databases retrieved 13 articles published until June 14, 2018. The most common clinical feature was a palpable rash (100% of patients), followed by joint pain (69.2%). Treatment response within 1-2 days was observed in 6 of 26 patients (23.1%) versus within 3-7 days in 17 patients (65.4%). Relapse after treatment discontinuation was reported in 17 patients (65.4%) but not in 3 patients (11.5 %). Four of the 26 patients (15.4%) reported adverse effects of dapsone including arthralgia (7.7%), rash (7.7%), and dapsone hypersensitivity syndrome (3.8%). Our findings suggest that dapsone may affect refractory IgA vasculitis. Multicenter randomized placebo-controlled trials are necessary to determine the standard dosage of dapsone at initial or tapering of treatment in IgA vasculitis patients and evaluate whether dapsone has a significant benefit versus steroids or other medications.