POS-581 PSEUDOPORPHYRIA CUTANEA TARDA IN HEMODIALYSIS

Abstract

Although dermatological manifestations in patients with end-stage renal disease (ESRD) are very common, such as pruritus, xerosis and pigmentation disorders, other skin disorders are quite rare such as pseudoporphyria cutanea tarda (PCT). It is a bullous photodermatosis with the same clinical and histological characteristics of porphyria cutanea tarda but in the absence of abnormalities in the metabolism of porphyrin. We report in this work 2 cases of ESRD patients who developed a PCT. We report in this work 2 cases of ESRD patients who developed a PCT in our hemodialysis (HD) unit. Case report 1: A 49-year-old man with a history of Marfan syndrome complicated by bilateral blindness and hypertension and ESRD secodary to an indeterminate nephropathy. He started HD in 2007. His renal failure was complicated by secondary hyperparathyroidism requiring 7/8 parathyroidectomy associated with total thyroidectomy because of the presence of a Tirads V classified thyroid nodule. After 10 years of HD, he developed a bullous dermatosis, a fragile skin with multiple erosions covered with brownish scabs. The lesions were localized in the photo-exposed areas : the face and the hands. He had no family history of porphyria, photosensitivity, or previous episodes of this dermatosis. The diagnosis retained was PCT of the dialysis patient. Ferritin was within the standards at this time. The treatment was based on N-acetylcysteine and photoprotective creams. The outcome was favorable with the disappearance of the bullous lesions leaving multiple atrophic and dyschromic scars without subsequent recurrence. Case report 2: A 48 year old man with a history of hypertension, diabetes, ESRD on indeterminate nephropathy. He started peritoneal dialysis in 2010. After 9 years, faced with recurrent peritonitis, he was switched to HD. Encapsulating peritonitis was diagnosed after starting HD, treated with tamoxifen and corticosteroids. After one year of HD, he developed bullous skin lesions with the presence of folliculitis on the back of his hand, arm and abdomen. Biologically, there was no biological inflammatory syndrome and the ferritin was at 212ng / ml. He was initially treated by local and systemic antibiotic therapy, but without improvement. The diagnosis subsequently retained was PCT of the dialysis patient and he was treated with N acetylcysteine, the prescription of photoprotection and antibiotic therapy for his surinfected lesions. The evolution was marked by the progressive regression of the lesions giving way to atrophic scars. There are multiple factors that can cause a push of PCT in dialysis patients, such as erythropoietin, iron overload, diuretics, but the cause may remain unknown. The main thing is to always think about this diagnosis in face of any bullous dermatosis and knowing how to stop the incriminating agent, prescribe photoprotection, in addition to N acetylcysteine having encouraging results in these patients.