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A case of porphyria cutanea tarda in association with idiopathic myelofibrosis and CREST syndrome

✍ Scribed by S.C. Lee; S.J. Yun; J-B. Lee; S-S. Lee; Y.H. Won

Publisher: John Wiley and Sons
Year: 2001
Tongue: English
Weight: 291 KB
Volume: 144
Category: Article
ISSN: 0007-0963
DOI: 10.1046/j.1365-2133.2001.03971.x

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✦ Synopsis

We report a 56-year-old Korean woman with porphyria cutanea tarda (PCT), showing multiple scarring bullae and hypertrichosis on sun-exposed areas of skin with postinflammatory hyperpigmentation. Sclerodermoid changes were also found on both hands, the face and neck. The patient had suffered from CREST syndrome, manifesting with Raynaud's phenomenon and sclerodactyly, for more than 15 years. Anticentromere antibody was positive. She had presented with splenomegaly 3 years before the development of PCT, and was diagnosed as having idiopathic myelofibrosis, based on bone marrow biopsy. In summary, she had had CREST syndrome for 15 years and later developed idiopathic myelofibrosis and PCT. This is the first reported case of PCT in association with idiopathic myelofibrosis and CREST syndrome.

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