A case of Dubin-Johnson syndrome with a pigmented neurofibroma |
Sung Han Yun, Seung Eun Lee, Nam Sik Kim, Jong In Kim, Ju Yeon Nam, Jung Im Jung, Ung Suk Yang |
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두빈-존슨증후군 환자에서 발생한 피부 신경섬유종 내 색소침착 1예 |
윤성한, 이승은, 김남식, 김종인, 남주연, 정정임, 양웅석 |
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Abstract |
Clinically, Dubin-Johnson syndrome is characterized by mild icterus without specific symptoms or signs. The icterus is so mild
that it is usually noted only during another illness, pregnancy, or the use of oral contraceptives. There is no pruritus in
Dubin-Johnson syndrome. The physical examination is usually normal, except for the icterus, although hepatosplenomegaly is seen
occasionally.
Histologically, the liver is normal, except for the presence of dense pigment making it appear black grossly. Pigmentation of tissues
other than the liver in patients with Dubin-Johnson syndrome has been reported only in a few cases.
We experienced a case of Dubin-Johnson syndrome with extrahepatic pigmentation in the skin with a neurofibroma in a
66-year-old man. (Korean J Med 76:S6-S9, 2009) |
Key Words:
Dubin-Johnson syndrome; Extrahepatic; Neurofibromatosis; Pigmentation |
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