Utility of Plasma Fluorometric Emission Scanning for Diagnosis of the First 2 Cases Reports of Variegate Porphyria: A Very Rare Type of Porphyrias in Thai
Leena Chularojanamontri MD*, Chanisada Tuchinda MD*, Chatchawan Srisawat MD, PhD**, Neelobol Neungton MD**, Sarawut Junnu BSc**, Surin Kanyok MSc**
Affiliation : * Department of Dermatology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok ** Department of Biochemistry, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok
Two Thai women who are siblings presented with a history of recurrent pruritic vesicles on dorsum of both hands and extensor surface of forearms where the sun-exposed areas are. The excoriated vesicles were healed with depressed scars. They had no previous history of intense abdominal pain, seizure, or psychiatric disorder. Urinary porphyrins were analyzed by High Performance Liquid Chromatography (HPLC). The level of coproporphyrin III was detected to be higher than the uroporphyrin level. Fluorescence emission scanning of both patients’ plasma was performed and demonstrated typical emission peak at 626 nm, that confirmed the diagnosis of variegate porphyria.
Keywords : Variegate porphyria, Plasma Fluorescence emission scanning, Thai
