An epidemic of porphyria cutanea tarda?

Case 1 A 43-year-old man first presented to the clinic of Wishard Memorial Hospital (WMH), serving Indianapolis Metropolitan Area, Indiana, on August 10, 1998 with symptoms of stinging, burning, and blistering bilaterally on the dorsum of the hands. His past medical history was significant for polys ubstance abuse, including alcohol. Physical examination revealed numerous e rosions on the dorsum of the hands and forearms, with crusts and excoriatio n, in addition to several small vesicles on the hands (Fig. 1). Hypertricho sis was noted in the temporal area. A sample of the patient's urine fluores ced reddish-pink under Wood's lamp exposure. Laboratory studies were positi ve for hepatitis C virus (HCV) antibodies (second generation enzyme-linked immunosorbent assay, ELISA), but negative for hepatitis B virus (HBV) marke rs. Hemoglobin was 18.2 g/dL and hematocrit 53%. Liver enzymes were elevate d with an aspartate aminotransferase (AST) of 214 units and alanine aminotr ansferase (ALT) of 297 units. A fractionated 24-h urine specimen collected on August 18, 1998 demonstrated a markedly elevated uroporphyrin of 4174 mu g/total volume (tv) (ref.: 3-30 mu g/tv) and a coproporphyrin of 123 mu g/ tv (ref: 0-155 mu g/tv). A diagnosis of porphyria cutanea tarda (PCT) and H CV was made, therapeutic phlebotomies were initiated, and the patient was r eferred to the Hepatology Section for follow-up. Case 2 A 40-year-old man first presented to our clinic on August 20, 1998 c omplaining of fragile skin, blister formation of the dorsum of the hands, h airy temporal area, and overall bronze appearance of the skin. The patient also described increased hair growth in the temporal area. The patient had hepatitis C (diagnosed in December 1993 by second generation ELISA), immuno globulin A (IgA) nephropathy requiring hemodialysis following 3 years of pe ritoneal dialysis, and polysubstance abuse (including alcohol). He had elev ated total plasma porphyrin of 2.4 mu g/dL (ref.: < 1.1 mu g/dL) and uropor phyrin of 1.1 mu g/dL (ref.: < 1.1 mu g/dL). With these findings on August 20, 1998, a diagnosis of PCT was made. Case 3 A 49-year-old man first presented to the WMH clinic on June 18, 1998 with blistering on the dorsum of the bilateral fingers and various pigment ations of the hands. He had anemia, polysubstance abuse (including alcohol) , and hemodialysis. A diagnosis of PCT was made on August 17, 1998 based on the findings of uroporphyrin 12.3 mu g/dL, heptacarboxyporphyrins 8.0 mu g /dL, and hexocarboxyporphyrins 2.4 mu g/dL (ref. all: < 1.1 mu g/dL). His H CV infection was established in June 1996 with a second generation ELISA. Case 4 A 32-year-old man first presented to the WMH clinic on September 4, 1998 upon noticing that he had shown similar symptoms and signs over the pa st year to his brother-in-law, who is Case 1 of this report. He was a heavy alcohol drinker. Tests performed on September 4, 1998 demonstrated HCV ant ibody, elevated AST/ALT, elevated uroporphyrins of 3253 mu g/tv, and coprop orphyrins of 735 mu g/tv in 24-h urine. A diagnosis of PCT was made and the rapeutic phlebotomies were initiated.