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 polysubstance abuse, including alcohol. Physical examination revealed numerous erosions on the dorsum of the hands and forearms, with crusts and excoriation, in addition to several small vesicles on the hands ( Fig. 1). Hypertrichosis was noted in the temporal area. A sample of the patient’s urine fluoresced reddish-pink under Wood’s lamp exposure. Laboratory studies were positive for hepatitis C virus (HCV) antibodies (second generation enzyme-linked immunosorbent assay, ELISA), but negative for hepatitis B virus (HBV) markers. Hemoglobin was 18.2 g/dL and hematocrit 53%. Liver enzymes were elevated with an aspartate aminotransferase (AST) of 214 units and alanine aminotransferase (ALT) of 297 units. A fractionated 24-h urine specimen collected on August 18, 1998 demonstrated a markedly elevated uroporphyrin of 4174 μg/total volume (tv) (ref.: 3–30μg/tv) and a coproporphyrin of 123 μg/tv (ref: 0–155μg/tv). A diagnosis of porphyria cutanea tarda (PCT) and HCV was made, therapeutic phlebotomies were initiated, and the patient was referred to the Hepatology Section for follow-up. Figure 1. Erosions and ulcerations on the dorsal aspect of the hands Download figure to PowerPoint Case 2 A 40-year-old man first presented to our clinic on August 20, 1998 complaining of fragile skin, blister formation of the dorsum of the hands, hairy 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), immunoglobulin A (IgA) nephropathy requiring hemodialysis following 3 years of peritoneal dialysis, and polysubstance abuse (including alcohol). He had elevated total plasma porphyrin of 2.4 μg/dL (ref.: <.1 μg/dL) and uroporphyrin of 1.1 μg/dL (ref.: <1.1 μg/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 pigmentations 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 μg/dL, heptacarboxyporphyrins 8.0 μg/dL, and hexocarboxyporphyrins 2.4 μg/dL (ref. all: <1.1 μg/dL). His HCV 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 past 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 antibody, elevated AST/ALT, elevated uroporphyrins of 3253 μg/tv, and coproporphyrins of 735 μg/tv in 24-h urine. A diagnosis of PCT was made and therapeutic phlebotomies were initiated.