Abstract
AbstractA 63-year-old black female patient with blisters and exulcerations on the face, neck, upper limbs, and subsequent evolution with hypochromic sclerotic areas and alopecia, is reported. Chronic hepatitis C and presence of high levels of porphyrins in urine were demonstrated. There was complete remission with the use of hydroxychloroquine, photoprotection, and treatment of hepatitis. Significant sclerodermoid involvement of the skin as a manifestation of porphyria cutanea tarda secondary to hepatitis C emphasizes the importance of diagnostic suspicion regarding skin manifestation in order to indicate the appropriate therapy, and to minimize the hepatic morbidity.
Highlights
In addition to the different clinical forms of cutaneous scleroderma, sclerodermiform disorders may be secondary to graft versus host disease, drugs, silica exposure, porphyrias, silicone implants, paraneoplastic manifestations, and chronic infections.[1,2,3,4,5,6,7]
The authors reported a case of porphyria cutanea tarda (PCT) - or chronic hepatic porphyria, with sclerodermiform lesions, secondary to chronic hepatitis C virus, with significant involvement of photoexposed areas.[6]
The diagnosis of PCT is given by clinical, histopathology, and porphyrin analysis
Summary
CASE REPORT A 63-year-old female patient reported blisters on the face, neck, upper, and lower limbs, which exuded and evolved as hypochromias and sclerotic areas one year ago, with no therapeutic response to topical corticosteroid therapy and phototherapy (NBUVB). There were exulcerations and hypochromic sclerotic plaques on the face, cervical region, upper, and lower limbs, as well as areas of frontal, temporal, and occipital alopecia (Figures 1 and 2). There was restriction on wrist movement and oral opening. She did not report Raynauld’s phenomenon or dysphagia, and nailfold capillaroscopy was normal. There was increase in hepatic enzymes (TGO 74 U/L - reference < 36 U/L, TGP 93 U/L - reference < 52 U/L), anemia (hemoglobin 10.8g/dL), and anti-HCV was positive. The urinary porphyrin analysis was positive and there was reddish fluorescence in urine exposed to Wood’s light (Figure 3)
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