Porfiria cutánea tarda: caso clínico / Porphyria cutanea tarda: case report
Rev. méd. Chile
; 146(8): 943-946, ago. 2018. graf
Artigo
em Espanhol
| LILACS
| ID: biblio-978780
Biblioteca responsável:
CL1.1
ABSTRACT
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.
Texto completo:
Disponível
Coleções:
Bases de dados internacionais
Base de dados:
LILACS
Assunto principal:
Porfiria Cutânea Tardia
/
Terapia de Reposição Hormonal
/
Didrogesterona
/
Estradiol
Limite:
Feminino
/
Humanos
Idioma:
Espanhol
Revista:
Rev. méd. Chile
Assunto da revista:
Medicina
Ano de publicação:
2018
Tipo de documento:
Artigo
País de afiliação:
Chile
Instituição/País de afiliação:
Hospital Puerto Montt Dr. Eduardo Schütz Schroeder/CL
/
Pontificia Universidad Católica de Chile/CL