Porphyria Cutanea Tarda

Noninflammatory blisters on sun-exposed sites, usually only the dorsal hands

Hypertrichosis, skin fragility

Associated liver disease

Elevated urine porphyrins

The disease is associated with ingestion of certain medications (eg, estrogens), and alcoholic liver disease or hepatitis C

Painless blistering and fragility of the skin of the dorsal surfaces of the hands

Facial hypertrichosis and hyperpigmentation

In patients with liver disease, hemosiderosis is often present

Differential diagnosis

• Pseudoporphyria: dialysis, medications (tetracycline, nonsteroidal anti-inflammatory drugs, voriconazole)

• Contact dermatitis

• Scabies

Urinary uroporphyrins are elevated two- to five-fold above coproporphyrins

There may be abnormal liver biochemical tests, evidence of hepatitis C infection, increased liver iron stores, and various hemochromatosis gene mutations

Phlebotomy without oral iron supplementation at a rate of 1 unit every 2–4 weeks will gradually lead to improvement

Hydroxychloroquine 200 mg orally twice weekly will increase the excretion of porphyrins, improving the skin disease

Deferasirox, an iron chelator can also improve porphyria cutanea tarda

Stopping all triggering medications and stopping alcohol consumption may lead to improvement

Most patients improve with treatment

Barrier sun protection with clothing is required for prevention