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Porphyria cutanea tarda (PCT) is a metabolic disorder resulted from reduced enzyme activity of uroporphyrinogen decarboxylase, which plays a role in heme-synthesis. Clinical manifestation primarily occurred due to excess porphyrin deposit on the skin and subcutaneous tissue. A 57-year-old Asian male, Balinese, seek for medical attention with chief complaint of wide erosion on his body after patient fell into a trench, the skin was peeled off when the patient was pulled out. Previously, patient had frequent blisters developed on sun exposed area of the body for the last 5 months. The blisters contained clear fluid and healed by scarring, hyperpigmentation and hypopigmentation. Photosensitivity and hair loss were present. Diagnosis of acquired PCT was achieved from anamnesis, clinical examination and confirmed by histopathological findings. Patient also suffered from chronic kidney disease (CKD) stage-2 and severe anemia. We managed this patient using conservative treatments involving gauze dressing impregnated with antibiotic, packed red blood cells transfusion and albumin transfusion. Patient showed improvement on skin lesions, however, was later diagnosed with MRSA infection and passed away during second week of hospitalization due to septic shock. PCT in patients with CKD faces a therapeutic challenge. Hydroxychloroquine and phlebotomy have been the standard treatment for PCT. However, in the presence of kidney disease, the use of chloroquine is not advisable and phlebotomy is precluded by severe anemia. Alternative approach is needed to manage PCT in the setting of CKD.
Irene Jessica Soputro has obtained her Medical degree from Faculty of Medicine, Universitas Indonesia and completed her Advance Medical Science program at University of Melbourne, Australia. She is completing her specialization in Department of Dermatovenereology, Faculty of Medicine, Udayana University, Indonesia.
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