Quiz: Blister in the sun

A 53-year-old Caucasian male with a background of haemochromatosis, fatty liver disease

and actinic keratosis presented with a one-year history of vesicles and bullae (see figure 1
right).
These blisters initially developed on his hands but subsequently progressed to involve sunexposed areas of the arms and feet.
He complained of fragile skin and slower wound healing. No oral involvement or significant
family history for any blistering disease was noted. He was drinking 30 units of alcohol per
week.
On examination, besides a generalised hypertrichosis, there were erosions and tense blisters
on the dorsal hands, arms and feet.
A provisional diagnosis of porphyria cutanea tarda (PCT) was made, with differential
diagnosis of pseudo-PCT and epidermolysis bullosa acquisita.
Lesional punch biopsies reported subepidermal blistering with a 'festooned' (undulating) base
and mild perivascular lymphocytic infiltrate.
Perilesional biopsy for direct immunofluorescence was negative. Furthermore, he had
elevated urine porphyrins with normal red blood cell porphyrin levels. These results are
consistent with PCT.
He was advised to wear sunscreen and cover his face and arms to reduce visible light
exposure. He was referred to haematology to arrange for phlebotomy and with a normal
G6PD, low-dose hydroxychloroquine is planned.

Professor Murrell is head of dermatology at St George Hospital and conjoint professor at

University of NSW. This article is co-authored by Asri Wijayanti, fourth-year medical
student, department of dermatology, St George Hospital; and Dr Cathy Zhao, clinical
research fellow, department of dermatology, St George Hospital and conjoint associate
lecturer at the University of NSW.

THE QUIZ
Q. Which of these are risk factors for PCT?
a) Prostate cancer, HIV, excessive alcohol intake.
b) Diabetes mellitus, hepatitis C virus, haemochromatosis.
c) Skin cancer, ischaemic heart disease, smoking.
d) Liver disease, stroke, renal disease.
A. The answer is b. PCT is strongly predisposed by diabetes mellitus (25%), hepatitis C virus
and haemochromatosis. Other predisposing factors include excessive alcohol intake,
smoking, HIV, liver disease and end-stage renal disease.
Q. Which of the following is a pattern seen in PCT?
a) Red blood cells uroporphyrin markedly elevated.
b) Plasma fluorescence positive.
c) Urine uroporphyrin markedly elevated.
d) Faeces protoporphyrin markedly elevated.
A. The answer is c. Normal red blood cells and faecal porphyrin levels are seen in PCT. Urine
fluorescence instead of plasma fluorescence is positive.
Q. Which statement regarding treatment is false?
a) High-dose hydroxychloroquine is an appropriate first-line treatment to induce
remission in all PCT patients.
b) Avoidance of ethanol and other drugs that could induce PCT is an important part of
management.
c) Phlebotomy is a commonly recommended treatment for PCT.
d) Regular application of a physical-blocker sunscreen is useful and largely
recommended.
A. The answer is a. High-dose hydroxychloroquine can exacerbate the disease and may
induce hepatic failure. Low-dose hydroxychloroquine can be used to induce remission of
PCT.