[Porphyria cutanea tarda. Case report]. / Porfiria cutánea tarda. Caso clínico.
Rev Med Chil
; 146(8): 943-946, 2018 Aug.
Article
en Es
| MEDLINE
| ID: mdl-30534876
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:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Porfiria Cutánea Tardía
/
Terapia de Reemplazo de Hormonas
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Didrogesterona
/
Estradiol
Límite:
Female
/
Humans
/
Middle aged
Idioma:
Es
Revista:
Rev Med Chil
Año:
2018
Tipo del documento:
Article
País de afiliación:
Chile