Porfiria cutánea tarda. Caso clínico

Porphyria cutanea tarda (PCT) is the most common type of porphyria: it is characterized by blistering lesions, erosions and crusts on the back of the hands, associated with photosensitivity and facial hypertrichosis. It is produced by acquired or hereditary deficiency of the enzyme UROD, fifth enzym...

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Autores principales:	Hermosilla B.,Nicolás, Toro,Gonzalo De, Molgó,Montserrat
Lenguaje:	Spanish / Castilian
Publicado:	Sociedad Médica de Santiago 2018
Materias:	Estrogens Porphyrias Porphyria Cutanea Tarda
Acceso en línea:	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872018000800943
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spelling	oai:scielo:S0034-988720180008009432018-10-18Porfiria cutánea tarda. Caso clínicoHermosilla B.,NicolásToro,Gonzalo DeMolgó,Montserrat Estrogens Porphyrias Porphyria Cutanea Tarda Porphyria cutanea tarda (PCT) is the most common type of porphyria: it is characterized by blistering lesions, erosions and crusts on the back of the hands, associated with photosensitivity and facial hypertrichosis. It is produced by acquired or hereditary deficiency of the enzyme UROD, fifth enzyme in the chain of production of the Heme group. This causes accumulation of porphyrins in the liver, which are subsequently mobilized to the skin, where lesions are generated by photosensitivity. This deficiency can be exacerbated by multiple causes. We report a 51-year-old female presenting with the characteristic dermal lesions described above, which disappeared when she discontinued her hormone replacement therapy with estradiol and dydrogesterone. Urinary and blood uroporphyrin and hexacarboxyl porphyrins were elevated and plasma ferritin was 479 ng/ml. Hormone replacement therapy was discontinued and phlebotomies were attempted but not tolerated by the patient. The dermic lesions have not relapsed.info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.146 n.8 20182018-08-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872018000800943es10.4067/s0034-98872018000800943
institution	Scielo Chile
collection	Scielo Chile
language	Spanish / Castilian
topic	Estrogens Porphyrias Porphyria Cutanea Tarda
spellingShingle	Estrogens Porphyrias Porphyria Cutanea Tarda Hermosilla B.,Nicolás Toro,Gonzalo De Molgó,Montserrat Porfiria cutánea tarda. Caso clínico
description	Porphyria cutanea tarda (PCT) is the most common type of porphyria: it is characterized by blistering lesions, erosions and crusts on the back of the hands, associated with photosensitivity and facial hypertrichosis. It is produced by acquired or hereditary deficiency of the enzyme UROD, fifth enzyme in the chain of production of the Heme group. This causes accumulation of porphyrins in the liver, which are subsequently mobilized to the skin, where lesions are generated by photosensitivity. This deficiency can be exacerbated by multiple causes. We report a 51-year-old female presenting with the characteristic dermal lesions described above, which disappeared when she discontinued her hormone replacement therapy with estradiol and dydrogesterone. Urinary and blood uroporphyrin and hexacarboxyl porphyrins were elevated and plasma ferritin was 479 ng/ml. Hormone replacement therapy was discontinued and phlebotomies were attempted but not tolerated by the patient. The dermic lesions have not relapsed.
author	Hermosilla B.,Nicolás Toro,Gonzalo De Molgó,Montserrat
author_facet	Hermosilla B.,Nicolás Toro,Gonzalo De Molgó,Montserrat
author_sort	Hermosilla B.,Nicolás
title	Porfiria cutánea tarda. Caso clínico
title_short	Porfiria cutánea tarda. Caso clínico
title_full	Porfiria cutánea tarda. Caso clínico
title_fullStr	Porfiria cutánea tarda. Caso clínico
title_full_unstemmed	Porfiria cutánea tarda. Caso clínico
title_sort	porfiria cutánea tarda. caso clínico
publisher	Sociedad Médica de Santiago
publishDate	2018
url	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872018000800943
work_keys_str_mv	AT hermosillabnicolas porfiriacutaneatardacasoclinico AT torogonzalode porfiriacutaneatardacasoclinico AT molgomontserrat porfiriacutaneatardacasoclinico
_version_	1718437021143269376

Porfiria cutánea tarda. Caso clínico

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