Childhood-onset porphyria cutanea tarda: successful therapy with low-dose hydroxychloroquine (Plaquenil).

Abstract

We describe a 4-year-old girl with a spontaneous blistering disorder that was consistent with porphyria cutanea tarda (PCT). There was no familial history of the disease or any obvious causative factors present. Oral hydroxychloroquine (3 mg/kg) was given twice weekly along with vitamin E (200 U/d) as an antioxidant. Within 6 weeks, marked decreased blistering occurred and by 12 weeks no blistering was evident. Despite clinical improvement and tolerance of hydroxychloroquine, urinary uroporphyrin, aspartate aminotransferase, and ferritin levels continued to rise reaching peak levels at 16 weeks of therapy. Near total biochemical remission was observed at 40 weeks and all therapy was discontinued at 60 weeks.

• Authors: A J Bruce, I Ahmed
• Journal: Journal of the American Academy of Dermatology (J Am Acad Dermatol) Vol. 38 Issue 5 Pt 2 Pg. 810-4 (May 1998) ISSN: 0190-9622 [Print] United States
• PMID: 9591792 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Antioxidants
• Antirheumatic Agents
• Uroporphyrins
• Vitamin E
• Hydroxychloroquine
• Ferritins
• Aspartate Aminotransferases

Topics

• Administration, Oral
• Antioxidants (therapeutic use)
• Antirheumatic Agents (administration & dosage, therapeutic use)
• Aspartate Aminotransferases (urine)
• Child, Preschool
• Female
• Ferritins (urine)
• Follow-Up Studies
• Humans
• Hydroxychloroquine (administration & dosage, therapeutic use)
• Porphyria Cutanea Tarda (drug therapy)
• Remission Induction
• Uroporphyrins (urine)
• Vitamin E (therapeutic use)