RESUMO
La acrodermatitis enteropática (AE) es una genodermatosis autosómica recesiva causada por la mutación del gen responsable de codificar a la proteína transportadora de Zinc (Zn) SLC39A4. A pesar de ser una rara enfermedad es de fácil manejo y gran relevancia clínica. Se caracteriza por la siguiente tríada: dermatitis acral y periorificial, diarrea y alopecia. Comunicamos un caso de presentación atípica en una lactante de 6 meses de edad con lesiones periorificiales y ampollas acrales que resolvió rápidamente con la terapia suplementaria con Zinc (AU)
Enteropathic acrodermatitis is an autosomal recessive genodermatosis caused by the mutation of the gene responsible for encoding the Zinc transporter protein SLC39A4. Despite being a rare disease, it is easy to manage and of great clinical relevance. It is characterized by the following triad: acral and periorificial dermatitis, diarrhea and alopecia. We report a case of atypical presentation in an almost 6-month-old infant with periorificial lesions and acral blisters that quickly resolved with supplemental Zinc therapy (AU)
Assuntos
Humanos , Feminino , Lactente , Zinco/deficiência , Acrodermatite/diagnóstico , Gastroenteropatias/diagnóstico , Acrodermatite/tratamento farmacológico , Sulfato de Zinco/uso terapêutico , Gastroenteropatias/tratamento farmacológicoRESUMO
Acroangiodermatitis is an angioproliferative disease usually related to chronic venous insufficiency, and it is considered a clinical and histological simulator of Kaposi's sarcoma (KS). Immunohistochemistry is the suitable method to differentiate between these two entities. It reveals the following immunostaining profile: immunopositivity with anti-CD34 antibody is restricted to the vascular endothelium in acroangiodermatitis, and diffuse in the KS (endothelial cells and perivascular spindle cells); immunopositivity with anti-HHV-8 only in KS cases. We report the case of an HIV seropositive patient without apparent vascular disease, who presented violaceous and brownish erythematous lesions on the feet, and whose histopathology and immunohistochemistry indicated the diagnosis of acroangiodermatitis.
Assuntos
Adulto , Humanos , Masculino , Acrodermatite/patologia , Soropositividade para HIV/patologia , Hepatite C/patologia , Sarcoma de Kaposi/patologia , Sífilis/patologia , Acrodermatite/tratamento farmacológico , Coinfecção/patologia , Diagnóstico Diferencial , Imuno-Histoquímica , Pele/patologiaRESUMO
Acrodermatitis enteropathica is an uncommon disease caused by hereditary or acquired zinc deficiency. It is characterized by a triad of alopecia, diarrhea and acraland periorificial dermatitis. It is treated with Zinc supplementation. We report a31-year-old indigent and drug addict female with a cutaneous-mucous syndromecharacteristic of acrodermatitis enteropathica. She had a positive clinical evolutionafter Zinc supplementation.
Assuntos
Adulto , Feminino , Humanos , Acrodermatite/patologia , Derme/patologia , Zinco/deficiência , Acrodermatite/diagnóstico , Acrodermatite/tratamento farmacológico , Biópsia , Diagnóstico Diferencial , Zinco/metabolismo , Zinco/uso terapêuticoAssuntos
Humanos , Feminino , Diarreia Infantil , Alopecia , Lactente , Zinco/sangue , Zinco , Acrodermatite/tratamento farmacológicoRESUMO
La acrodermatitis enteropática es una enfermedad, autosómica recesiva poco frecuente, que se presenta en los primeros meses de vida. Se comunican dos pacientes que presentaron alopecia, dermatitis acral, alteraciones digestivas, además de irritabilidad e infecciones recurrentes. Los estudios de laboratorio confirman niveles bajos de zinc y de fosfatasa alcalina. La terapia sustitutiva con sulfato de zinc produjo una rápida mejoría
Assuntos
Humanos , Masculino , Feminino , Lactente , Acrodermatite/diagnóstico , Transtornos da Nutrição do Lactente/complicações , Transtornos da Nutrição do Lactente/tratamento farmacológico , Zinco/deficiência , Acrodermatite/tratamento farmacológico , Acrodermatite/etiologia , Diagnóstico Diferencial , Zinco/sangue , Zinco/uso terapêuticoRESUMO
The therapeutic effect of orally administered zinc sulphate was evaluated in 16 patients with acrodermatitis enteropathica [AE]. Zinc serum levels in these patients and apparently healthy close relatives to patients [the control group] were estimated by atomic absorption spectrophotometric technique. The present study was found a low zinc serum levels in all patients with AE compared to the control group [p<0.001]. Oral zinc sulphate therapy resulted in rapid and complete clinical response. The study concluded that zinc therapy could be used as a diagnostic test as the basic treatment of AE
Assuntos
Acrodermatite/tratamento farmacológicoRESUMO
Se describen los hallazgos clínicos y de laboratorio en un niño de 26/12 años de edad con acrodermatitis enteropática y la respuesta favorable a la terapia con cinc oral. Las manifestaciones clínicas remitieron rápidamente y después de un año de tratamiento se encuentra libre de síntomas y con incremento considerable del peso y la talla