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By Kirkland Lau, DO, and Stephen M. Schleicher, MD
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CASE:
A 50-year-old woman presents with a 10-month history of skin fragility and blister formation affecting her hands. She has also noticed bruising and scarring after minimal trauma. The affected areas are occasionally pruritic, and topical therapy with hydrocortisone cream has proven ineffectual. Several years ago, the patient was diagnosed with hepatitis C, but she is not currently on any oral medications. She states that since completing antiviral therapy her viral load is no longer detectable. Examination of her hands reveals scattered denuded patches and atrophic scars. No other cutaneous abnormalities are noted.
WHAT IS YOUR DIAGNOSIS? |
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Porphyria cutanea tarda should be suspected given the clinical presentation and history of hepatitis C. The diagnosis can be confirmed by a markedly elevated uroporphyrin level in a 24-hour urine collection. The cutanea tarda subset is the most common type of porphyria and results from a deficiency of hepatic uroporphyrinogen decarboxylase activity. The disease is most commonly induced by alcohol and is linked to hepatitis C infection. Clinical features include bullae in sun-exposed areas, skin fragility, facial hypertrichosis, sclerodermoid features, and milia. The preferred treatment is phlebotomy to lower serum iron levels. Ancillary measures include sun avoidance and abstaining from alcohol. |
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Dr. Lau is an associate with a division of DermDx Centers for Dermatology in Sinking Spring, Pennsylvania. Dr. Schleicher is director of DermDx Centers and a clinical instructor of dermatology at the Philadelphia College of Osteopathic Medicine, at Kings College in Wilkes-Barre, Pennsylvania, and at Arcadia University in Glenside, Pennsylvania. He is also a member of the EMERGENCY MEDICINE editorial board.
Emerg Med 39(9):55-6, 2007
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