Successful Chloroquine Treatment of Porphyria Cutanea Tarda in
HIV Patient on Hemodialysis
Joanna Yun, Steven Zivich, Geoff Lim, RoseMary Harris
Department of Family Medicine, Drexel University College of Medicine, Philadelphia, PA
.
Case Report
Background
References
A 52 year old Caucasian male with HIV/AIDS
recently restarted on antiretroviral therapy,
Hepatitis C with cirrhosis of the liver resistant
to Pegasus/ribavirin therapy, and end-stage
renal disease on hemodialysis for past 18
months; presents to his primary care doctor
with complaints of new hand lesions that
developed over the last 2 months. The patient
is heterosexual male, 40 pack year tobacco
smoking history, alcohol abuse and has a
history of heroin dependence.
Porphyria cutanea tarda (PCT) is a metabolic disease characterized by a decrease in the enzyme activity of
uroporphyrinogen decarboxylase (UROD), the 5th enzyme in the heme biosynthesis pathway.(1,2) Reduction
of UROD activity results in accumulation of uroporphyrin and other highly carboxylated porphyrins in the
skin and liver, resulting in delayed phototoxic skin reactions and asymptomatic chronic hepatopathy.(3) The
accumulated byproducts are photosensitive, which results in much of the clinical presentation.(4)
1. Singal AK, Kormos-Hallberg C, Lee C, et al. Lowdose hydroxychloroquine is as effective as
phlebotomy in treatment of patients with porphyria
cutanea tarda. Clin Gastroenterol Hepatol.
2012;10(12):1402-9. doi:10.1016/j.cgh.2012.08.038.
2. Munoz-Santos C, Guilabert A, Moreno N, ToFigueras J, Badenas C, Darwich E, Herrero C.
Familial and sporadic porphyria cutanea tarda:
clinical and biochemical features and risk factors in
152 patients. Medicine. 2010;89: 69-74.
3. Balwani M, Desnick RJ. The porphyrias; advances
in diagnosis and treatment. Blood. 2012;120(23):
4496-504.
4. Huang Y-C, Wang C-C, Sue Y-M. Porphyria
cutanea tarda in a hemodialysis patient. QJM.
2013;106(6):591-2. doi:10.1093/qjmed/hcs115.
5. Quansah R, Cooper CJ, Said S, Bizet J, Paez D,
Hernandez GT. Hepatitis C- and HIV-induced
porphyria cutanea tarda. Am J Case Rep.
2014;15:35-40. doi:10.12659/AJCR.889955.
6. Markova A, Lester J, Wang J, Robinson-Bostom L.
Diagnosis of common dermopathies in dialysis
patients: a review and update. Semin Dial.
2012;25(4): 408-18.
7. Mansourati F, Stone V, Mayer K. Porphyria
cutanea tarda and HIV/AIDS: a review of
pathogenesis, clinical manifestations and
management. Int J STD AIDS. 1999;10:51-56.
8. Poh-Fitzpatrick MB. Porphyria cutanea tarda:
treatment options revisited. Clin Gastroenterol
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doi:10.1016/j.cgh.2012.09.005.
9. Shieh S, Cohen JL, Lim HW. Management of
porphyria cutanea tarda in the setting of chronic
renal failure: A case report and review. J Am Acad
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doi:10.1067/mjd.2000.105504.
10. Tarwater K, Misra S, Misra M. Type I (sporadic)
porphyria cutanea tarda in a hemodialysis patient: a
case report. Hemodial Int. 2008;12 Suppl 2(Figure
3):S38-42. doi:10.1111/j.1542-4758.2008.00323.x.
Lesions were excoriated with multiple blisters
and crusting, localized only to the hands. The
patient had no other complaints. Lesions
were cultured for HSV, which later resulted
negative. Patient was discharged on
Triamcinolone 0.1% cream, Acyclovir 400mg
and Silvadene cream daily.
Patient returns 3 months later, hands lesions
remained unchanged from antivirals and
topical therapy. At this time patient’s CD4 171
and HIV VL 70.
Patient was evaluated by dermatology,
underwent skin biopsy and was diagnosed
with porphyria cutanea tarda (PCT).
Patient did not tolerate the initial therapy of
prednisone 20mg due to psychiatric side
effects. Phlebotomy therapy was initiated,
however after 2 months of intermittent
compliance of phlebotomy there was no affect
on the hand lesions.
Due to poor response to previous therapies
and lack of alternative options, He was placed
on Chloroquine Phosphate despite literature
stating poor efficacy in patients on
hemodialysis. After 8 weeks of Chloroquine
therapy, the patient had almost completely
resolution of rash.
PCT is often divided into two types. Type I is acquired deficiency in UROD with common inciting factors of
type I are: Hepatitis C infection, alcohol abuse, hemochromatosis, HIV, smoking, estrogen use, hemodialysis
(HD) and chlorinated polycyclic aromatic hydrocarbons; which our patient had 5 risk factors.(5, 6, 7) Type 2
results from an autosomal dominant genetic mutation of UROD.
Treatments include photoprotection and avoidance of triggers, serial phlebotomies and antimalarials
chloroquine phosphate and hydroxychloroquine sulfate.(8) Antimalarials such as chloroquine form a watersoluble complex with the porphyrins and are excreted by the kidneys.(9) HD patients do not benefit from this
treatment as chloroquine-porphyrin complexes are not efficiently cleared during dialysis.(9) The first
reported case of antimalarial therapy effectively treating a patient was PCT in 2008 with serial phlebotomies
and low-dose hydrochloroquine.(10) This is the first case of a HD patient effectively being treated with
chloroquine therapy without phlebotomy.
Before
After