Document Detail

Pseudoporphyria: a clinical and biochemical study of 20 patients.
MedLine Citation:
PMID:  11357795     Owner:  NLM     Status:  MEDLINE    
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 abnormalities. The major presenting clinical features were blistering in 19 patients (95%), scarring in 14 (70%), photosensitivity in 13 (65%), skin fragility in 13 (65%), and milia in 8 (40%). Histologically, of 17 patients tested, 12 (71%) had classic findings of subepidermal separation with festooning of dermal papillae. None of the 11 patients tested had hepatitis 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.
C F Schanbacher; E R Vanness; M S Daoud; A Tefferi; W P Su
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Mayo Clinic proceedings     Volume:  76     ISSN:  0025-6196     ISO Abbreviation:  Mayo Clin. Proc.     Publication Date:  2001 May 
Date Detail:
Created Date:  2001-05-18     Completed Date:  2001-06-07     Revised Date:  2013-12-13    
Medline Journal Info:
Nlm Unique ID:  0405543     Medline TA:  Mayo Clin Proc     Country:  United States    
Other Details:
Languages:  eng     Pagination:  488-92     Citation Subset:  AIM; IM    
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MeSH Terms
Medical Records Systems, Computerized
Middle Aged
Porphyrias / metabolism*,  pathology
Porphyrins / blood*
Retrospective Studies
Reg. No./Substance:

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