Résumé
As poroceratoses compreendem um grupo de doenças da queratinização epidérmica, hereditárias ou adquiridas, caracterizadas histologicamente pela presença de lamela cornoide. A variante clínica designada por poroceratose superficial disseminada tem sido descrita na literatura, associada a estados de imunossupressão, como transplantação de órgãos, terapêuticas imunossupressoras e infecções. A sua associação a neoplasias sólidas foi raramente descrita na literatura, estando publicados apenas 5 casos. Descrevemos o caso clínico de um paciente que desenvolveu, subitamente, lesões de poroceratose superficial disseminada, concomitantemente ao diagnóstico de um colangiocarcinoma.
Porokeratosis refers to a group of hereditary or acquired disorders of epidermal keratinization and is characterized histologically by the presence of a cornoid lamella. The clinical variant referred to as disseminated superficial porokeratosis has been described in the literature in association with immunosuppressive conditions that include organ transplant, infections and immunosuppressive treatments. The association of disseminated superficial porokeratosis with solid organ malignancies has seldom been described, only 5 such cases having been published. The present report refers to a patient with lesions of disseminated superficial porokeratosis of sudden onset shortly before diagnosis of a cholangiocarcinoma.
Sujets)
Sujet âgé de 80 ans ou plus , Humains , Mâle , Conduits biliaires intrahépatiques , Tumeurs des canaux biliaires/complications , Cholangiocarcinome/complications , Syndromes paranéoplasiques/étiologie , Porokératose/étiologie , Porokératose/anatomopathologieRésumé
O termo woolly hair, ou cabelo lanoso, refere-se a uma variante anormal de cabelo, que pode ser parcial ou generalizado. As variantes de woolly hair generalizado são raras e podem ocorrer isoladamente ou associadas a outras alterações cutâneas e extracutâneas. Desta forma, nestes doentes, é necessário a exclusão de certos síndromes, como o de Noonan e cardiofaciocutâneo (CFC), que cursam com importante morbilidade e mortalidade. É descrito o caso clínico de uma criança com woolly hair generalizado e queratose pilar, mas que, após avaliação dos vários órgãos e sistemas, não foi encontrada qualquer alteração ou característica associada a esses síndromes.
The term woolly hair, or tightly curled hair, refers to a structural anomaly of scalp hair, which can be partial or generalized. Generalized woolly hair variants are rare and can occur in isolation or in association with other cutaneous and extra-cutaneous abnormalities. Thus, it is important to dismiss in these patients the diagnosis of syndromes such as the Noonan syndrome and Cardiofaciocutaneous syndrome, which have high morbidity and mortality rates. We report the clinical case of a 7 year-old boy with generalized woolly hair and keratosis pilaris. After an evaluation of organs and systems, no alteration or characteristics associated with these syndromes were found.
Sujets)
Enfant , Humains , Mâle , Poils/malformations , Poils/anatomopathologieRésumé
Pemphigus vegetans is a rare variant of pemphigus vulgaris characterized by vegetating plaques in the flexural regions. The coexistence of pemphigus vegetans and internal neoplasm is rare, being described only in four cases in the literature. We report the case of a patient with a typical skin eruption of pemphigus vegetans, who was detected with colonic cancer.