RÉSUMÉ
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.
Sujet(s)
Humains , Femelle , Adulte d'âge moyen , Porphyrie cutanée tardive/diagnostic , Porphyrie cutanée tardive/induit chimiquement , Hormonothérapie substitutive/effets indésirables , Dydrogestérone/effets indésirables , Oestradiol/effets indésirablesRÉSUMÉ
El síndrome de Sweet es una dermatosis inflamatoria poco común, que se ha asociado a tumores malignos, principalmente de tipo hematológico. Presentamos un caso clínico de síndrome de Sweet asociado con una rara neoplasia pancreática, siendo uno de los pocos casos reportados en la literatura médica acerca de esta asociación.
Sweet's syndrome is an uncommon inflammatory dermatosis, which has been associated with malignant tumors, mainly of hematological type. We report a clinical case of Sweet syndrome associated with a rare pancreatic neoplasm, which is one of the few cases reported in the medical literature about this association.
Sujet(s)
Humains , Mâle , Sujet âgé de 80 ans ou plus , Tumeurs du pancréas/anatomopathologie , Syndrome de Sweet/anatomopathologie , Glucagonome/anatomopathologie , Tumeurs du pancréas/imagerie diagnostique , BiopsieRÉSUMÉ
Background:Overexpression/amplification of the HER2 gene in advanced gastric cancer is a predictor of response to adjuvant therapy with monoclonal antibodies.Aim: To determine the frequency of HER2 gene overexpression and amplificationin advanced gastric cancer. Material and Methods: One hundred nine advancedgastric cancer biopsy specimens, from 76 men and 33 women aged 67 ± 14 and 62± 12 years respectively, were selected. Three histological patterns (diffuse, intestinaland mixed) were recognized. Automated immunohistochemistry was performedwith monoclonal c-erbB-2 (NCL-356) Novocastra. Fluorescent in situ hybridization (FISH) for HER2 was performed in positive cases. Results: In 39% of cases,immunohistochemical staining was negative. It was 1+, 2+ and 3+ positive in 15,36 and 11% of cases, respectively. It was positive in 16% and 3% of intestinal typeand mixed carcinomas, respectively. It was negative in all diffuse carcinomas. FISHwas performed in 39 (2 +) cases and in 11 (3 +) cases. The gene amplification waspositive in two (2 +) and 11 (3 +) cases (11.9%). The overall concordance betweenimmunohistochemical staining and in situ hybridization was 85%. Conclusions: Inadvanced gastric cancer, HER2 gene overexpression or amplification was observed in11% and 12% of cases, respectively.