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

We report a 56-year-old Korean woman with porphyria cutanea tarda (PCT), sh owing multiple scarring bullae and hypertrichosis on sun-exposed areas of s kin with postinflammatory hyperpigmentation. Sclerodermoid changes were als o found on both hands, the face and neck. The patient had suffered from CRE ST syndrome, manifesting with Raynaud's phenomenon and sclerodactyly, for m ore 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 my elofibrosis and PCT. This is the first reported case of PCT in association with idiopathic myelofibrosis and CREST syndrome.