Objective: To examine prospectively porphyrin metabolism in a human im
munodeficiency virus (HIV)-positive population. Setting: Specialist re
ferral unit at the Department of Genitourinary Medicine, St James's Ho
spital, Dublin, Ireland. Patients: Twenty-eight men and 5 women (age r
ange, 18-35 years). Twenty-nine were current or previous intravenous d
rug abusers. Four were thought to have sexually acquired HIV infection
. All had a history of acquired immunodeficiency syndrome-defining ill
nesses. The patients were selected as a consecutive sample from the in
patient department. Eligibility criteria were cooperation with urine a
nd stool collection and confirmed HIV seropositivity. The patients wer
e matched to 2 groups: 1 with normal results of porphyrin studies and
the other with abnormal findings from porphyrin studies. Intervention:
None. Main Outcome Measures: Plasma, urine, and stool porphyrin excre
tion patterns. Results: Of the 33 patients in the study, 13 (40%) had
increased urinary porphyrin excretion. All but 2 of these patients wer
e seropositive for hepatitis C virus. No study patient had clinical ev
idence of porphyria. Four patients (12%), however, had urine and stool
porphyrin excretion patterns that were classic for porphyria cutanea
tarda. All 4 of these patients were hepatitis C virus-positive. Patien
ts with porphyrinuria had a greater degree of immunosuppression (P=.00
9). Conclusions: Commonly, porphyrin metabolism is abnormal in persons
with established HIV infection. Hepatitis C may contribute to abnorma
l porphyrin metabolism. An unexpected number of patients studied had p
orphyrin excretion patterns that were characteristic of porphyria cuta
nea tarda, and all of these were hepatitis C virus-positive. A diagnos
is of porphyria cutanea tarda, especially in a young patient, should p
rompt investigation for underlying HIV and hepatitis C virus infection
s. Dermatologists should be aware of the infectious risk associated wi
th the vesicles and erosions in theses patients. Porphyrin studies sho
uld be performed in any patient with HIV and photosensitivity.