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3.
Rev. esp. pediatr. (Ed. impr.) ; 65(6): 551-552, nov.-dic. 2009.
Artigo em Espanhol | IBECS | ID: ibc-92225

RESUMO

El lupus neonatal es un síndrome raro que afecta a recién nacidos y lactantes, que se define por la presencia e autoanticuerpos maternos y características clínicas, como lesiones cutáneas típicas, bloqueo cardíaco completo, miocardiopatía, alteraciones hematológicas y hepatobiliares. Presentamos un lactante de 5 semanas derivado desde Atención Primaria por lesiones cutáneas anulares en tronco, cuero cabelludo y cara. Como antecedentes destaca en la mare afecta de síndrome Overlap (esclerosis sistémica, lupus y miositis). Se inicia estudio confirmándose el diagnóstico de lupus neonatal mediante punch biopsia y ANA moteado positivo con anti SSA-Ro y anti RNP positivos (AU)


Neonatal Lupus Erythematosus is a rare disroder that affects newborns and infants and it is caused by mother´s autoantibodies and several clinical features as typical skin rash, congenital complete heart block, miocardiapathies, haematoligical and hepatobiliary diseases. We attended a 5 weeks male infant, who was derived from the primary care center because of an annular skin rash on trunk, scalp and face. There was maternal history of Overlap syndrome (systemic sclerosis, lupus and myosistis). Diagnosis of neonatal Lupus was made by the skin biopsy and an ANAs with autoantibodies against Ro and U1-ribonucleoprotein (RNP) (AU)


Assuntos
Humanos , Masculino , Recém-Nascido , Lúpus Eritematoso Cutâneo/congênito , Trombocitopenia/congênito , Autoanticorpos/isolamento & purificação , Bloqueio Cardíaco/congênito , Escleroderma Sistêmico/complicações , Miosite/complicações , Lúpus Eritematoso Sistêmico/complicações
4.
An Med Interna ; 24(5): 239-41, 2007 May.
Artigo em Espanhol | MEDLINE | ID: mdl-17907890

RESUMO

Cowden's disease is a rare genodermatosis that is characterized for multiple cutaneous and visceral hamartoma . Lhermitte-Duclos's disease is a cerebelous lesion that consists in the displasic enlargement of the cerebelous circumvolution. It's incluyed in phacomatosis and usually presents associated to Cowden's disease, tuberous sclerosis and overlap syndromes.A 56 years old man, diagnosed in Dermatology with Cowden's disease ten years ago. In the extension study, he had hamartoma intestinal polip, esophagic glucogenic acanthosis and two solid thyroid nodules. The craneal TC didn't show significant alteration. Ten years after diagnosis a cerebral magnetis resonance was performed for intense cephalea, and it was found a bad-defined mass in right cerebelous hemisphere without contrast captation, compatible with cerebelous glangliocytoma.


Assuntos
Neoplasias Cerebelares/complicações , Ganglioneuroma/complicações , Síndrome do Hamartoma Múltiplo/complicações , Neoplasias Cerebelares/diagnóstico , Feminino , Ganglioneuroma/diagnóstico , Síndrome do Hamartoma Múltiplo/diagnóstico , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade
5.
Rev. esp. pediatr. (Ed. impr.) ; 63(3): 245-247, mayo-jun. 2007.
Artigo em Espanhol | IBECS | ID: ibc-126992

RESUMO

El eritema discrómico Perstans o dematosis cenicienta es una dermatosis idiopática adquirida, caracterizada por la aparición de manchas gris pizarra en el tronco, extremidades y cuello. De etiología desconocida, su prevalencia es mayor entre la población latinoamericana (AU)


The Erythema Dischromicum Perstans (ashy dermatosis) is an idiopathic acquired blue-gray macular hypermelanosis, usually affects the trunk, arms, neck and face. The etiology of the ashy dermatosis remains unknown and it´s more common in Latin America (AU)


Assuntos
Humanos , Eritema/diagnóstico , Nevo/diagnóstico , Diagnóstico Diferencial , Distribuição por Etnia
6.
An. med. interna (Madr., 1983) ; 24(5): 239-241, mayo 2007. ilus
Artigo em Es | IBECS | ID: ibc-056100

RESUMO

La enfermedad de Cowden es una rara genodermatosis que se caracteriza por la aparición de multiples hamartomas tanto a nivel cutaneomucoso como visceral. Es una lesión cerebelosa que consiste en el engrosamiento displásico de las circunvoluciones cerebelosas. Se incluye dentro de las facomatosis y suele presentarse junto a la enfermedad de Cowden, la esclerosis tuberosa o síndromes de solapamiento. Presentamos un paciente de 56 años diagnosticado en la consulta de Dermatología de enfermedad de Cowden hace 10 años. En el estudio de extensión se le diagnostica de poliposis intestinal hamartomatosa, acantosis glucogénica esofágica y se encuentran dos nódulos sólidos tiroideos. A los 10 años del diagnóstico se realiza resonancia magnética nuclear cerebral por aparición de cefalea encontrando una masa mal definida en hemisferio cerebeloso derecho compatible con gangliocitoma displásico cerebeloso


Cowden’s disease is a rare genodermatosis that is characterized for multiple cutaneus and visceral hamartoma . Lhermitte-Duclos´s disease is a cerebelous lesion that consists in the displasic enlargement of the cerebelous circumvolution. It´s incluyed in phacomatosis and ussually presents associated to Cowden’s disease, tuberous sclerosis and overlap syndromes. A 56 years old man, diagnosed in Dermatology with Cowden’s disease ten years ago. In the extension study, he had hamartoma intestinal polip, esophagic glucogenic acanthosis and two solid thyroid nodules. The craneal TC didn´t show significant alteration. Ten years after diagnosis a cerebral magnetis resonance was performanced for intense cephalea, and it was found a bad-defined mass in right cerebelous hemisphere without contrast captation, compatible with cerebelous glangliocytoma


Assuntos
Masculino , Adulto , Pessoa de Meia-Idade , Humanos , Síndrome do Hamartoma Múltiplo/complicações , Polipose Intestinal/complicações , Língua/patologia , Síndrome do Hamartoma Múltiplo/diagnóstico , Polipose Intestinal/diagnóstico , Espectroscopia de Ressonância Magnética , Língua/lesões
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