TY - JOUR
T1 - Pseudoporphyria
T2 - A clinical and biochemical study of 20 patients
AU - Schanbacher, Carl F.
AU - Vanness, Erin R.
AU - Daoud, Mazen S.
AU - Tefferi, Ayalew
AU - Daniel, W. P.
PY - 2001
Y1 - 2001
N2 - • Objective: To describe the clinical and laboratory findings in patients with pseudoporphyria. • Patients and Methods: This retrospective review identified 261 patients with either porphyrin metabolism abnormalities or pseudoporphyria who were seen at the Mayo Clinic in Rochester, Minn, between 1992 and 1996. All patients with documented porphyria cutanea tarda (PCT), noncutaneous porphyrias, or variegate porphyria were excluded. • Results: Twenty patients had active cutaneous lesions resembling, PCT with no diagnostic laboratory abnormalihepatitis B or C. In all 20 patients, porphyrin profiles were nondiagnostic. Of 16 patients for whom follow-up was available, 11 reported persistent symptoms for a mean of 2.5 years after evaluation. Five patients were free of symptoms 1 week to 6 months after discontinuation of the presumed offending agent. • Conclusion: Pseudoporphyria mimics the cutaneous symptoms of PCT in the setting of normal or near-normal porphyrin levels in the serum, urine, or stool. Despite efforts to discontinue an offending medication, symptoms may persist indefinitely.
AB - • Objective: To describe the clinical and laboratory findings in patients with pseudoporphyria. • Patients and Methods: This retrospective review identified 261 patients with either porphyrin metabolism abnormalities or pseudoporphyria who were seen at the Mayo Clinic in Rochester, Minn, between 1992 and 1996. All patients with documented porphyria cutanea tarda (PCT), noncutaneous porphyrias, or variegate porphyria were excluded. • Results: Twenty patients had active cutaneous lesions resembling, PCT with no diagnostic laboratory abnormalihepatitis B or C. In all 20 patients, porphyrin profiles were nondiagnostic. Of 16 patients for whom follow-up was available, 11 reported persistent symptoms for a mean of 2.5 years after evaluation. Five patients were free of symptoms 1 week to 6 months after discontinuation of the presumed offending agent. • Conclusion: Pseudoporphyria mimics the cutaneous symptoms of PCT in the setting of normal or near-normal porphyrin levels in the serum, urine, or stool. Despite efforts to discontinue an offending medication, symptoms may persist indefinitely.
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U2 - 10.4065/76.5.488
DO - 10.4065/76.5.488
M3 - Article
C2 - 11357795
AN - SCOPUS:0035036507
SN - 0025-6196
VL - 76
SP - 488
EP - 492
JO - Mayo Clinic proceedings
JF - Mayo Clinic proceedings
IS - 5
M1 - 62914
ER -