Case for diagnosis. Sclerodermiform manifestations of porphyria cutanea tarda secondary to hepatitis C

Abstract

CASE REPORTA 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). FIgure 1: A. Diffuse sclerotic hypochromic plaques on the face with alopecia. B. Detail of the dyschromic alterations, exulcerated lesion on the forehead, alopecia of the eyebrows and temporal margins Case for diagnosis. Sclerodermiform manifestations of porphyria cutanea tarda secondary to hepatitis C 481 An Bras Dermatol. 2019;94(4):479-81.Abstract: A 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.

Immunologic and nonimmunologic sclerodermal skin conditions - review

“…Rodisco and colleagues identified the presence of Raynaud’s phenomenon and cutaneous sclerosis associated with colorectal carcinoma in a 60-year-old patient. They note that cutaneous sclerosis could be a paraneoplastic manifestation ( 28 , 29 ). This aspect was also taken into account in the case of the Mekel diverticulum with neoplastic risk ( 30 ).…”

“…Bleomycin produces sclerodactyly with scleredema, cutaneous sclerosis, Raynaud’s phenomenon and hyperpigmentation. Also, Docetaxel induces scleroderma-like lesions, edema of the subcutaneous tissue in the limbs, erythematous spots with varying intensity from pale red to purplish, and even bullous lesions ( 29 , 50 ).…”

“…Calado and colleagues reported the case of an elderly black patient with sclerodermal lesions on the background of late cutaneous porphyria secondary to viral hepatitis C. The level of porphyrin in the urine was very high. Exulcerative and vesicular skin lesions on the face, neck, and lower limbs progressed to sclerosis, hypopigmentation and alopecia and completely recovered after administration of hepatoprotectants, photoprotectors, and hydroxychloroquine immunosuppression ( 29 , 50 ). Scleroderm lesions have been identified in other cases of late cutaneous porphyria without the presence of hepatic viral infection, sometimes in the presence of hemochromatosis and appear to be associated with female sex ( 3 , 29 ).…”

“…Exulcerative and vesicular skin lesions on the face, neck, and lower limbs progressed to sclerosis, hypopigmentation and alopecia and completely recovered after administration of hepatoprotectants, photoprotectors, and hydroxychloroquine immunosuppression ( 29 , 50 ). Scleroderm lesions have been identified in other cases of late cutaneous porphyria without the presence of hepatic viral infection, sometimes in the presence of hemochromatosis and appear to be associated with female sex ( 3 , 29 ). Histopathological examination showed excessive collagen deposition in the hardened and infiltrated inflammatory dermis with a predominance of mast cells ( 29 ).…”

“…Scleroderm lesions have been identified in other cases of late cutaneous porphyria without the presence of hepatic viral infection, sometimes in the presence of hemochromatosis and appear to be associated with female sex ( 3 , 29 ). Histopathological examination showed excessive collagen deposition in the hardened and infiltrated inflammatory dermis with a predominance of mast cells ( 29 ).…”