Acrodermatitis enteropática ampollosa / Bullous Acrodermatitis Enteropathica

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)

Acroangiodermatitis (pseudo-Kaposi's sarcoma) in an HIV sero-positive patient with syphilis and hepatitis C virus coinfection: clinical and dermatopathological features.

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.

Acroangiodermatitis (pseudo-Kaposi's sarcoma) in an HIV sero-positive patient with syphilis and hepatitis C virus coinfection: clinical and dermatopathological features

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.

Acrodermatitis enteropática: caso clínico y revisión de la literatura / Acrodermatitis enteropathica: report of one case

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.

[Acrodermatitis enteropathica: report of one case]. / Acrodermatitis enteropática: caso clínico y revisión de la literatura.

Acrodermatitis enteropathica is an uncommon disease caused by hereditary or acquired zinc deficiency. It is characterized by a triad of alopecia, diarrhea and acral and periorificial dermatitis. It is treated with Zinc supplementation. We report a 31-year-old indigent and drug addict female with a cutaneous-mucous syndrome characteristic of acrodermatitis enteropathica. She had a positive clinical evolution after Zinc supplementation.

Acrodermatitis enteropática: reporte de un caso y revisión de la literatura / Enteropathic acrodermatitis: a case report and a revision of literarure

En este artículo se aborda el contexto de las amenazas, desastres y la comunicación de riesgo desde un prisma integrador y actual. Se comentan algunas definiciones y enfoques, se comentan las tendencias internacionales sobre la incorporación de los niños y adolescentes a la gestión de riesgos. Se hacen reflexiones críticas sobre las dificultades que aún existen en la prevención, comunicación y reducción de los riesgos y amenazas potenciales en las actuales condiciones de cambio climático(AU)

This article reffers to the context of threats, disasters and risk communication since an integrated and current prism. There are comments of some definitions and the international tendencies about the incorporation of children and teenagers to the risk management. There are critical reflections about the difficulties that still occur in the prevention, communication and reduction of risk and potencial threats in the current conditions of the climatic change

An infant with acrodermatitis continua of Hallopeau: successful treatment with thalidomide and UVB therapy.

Acrodermatitis Continua of Hallopeau is a rare, chronic, recurrent disorder classified as a form of pustular psoriasis, and most cases affect one or two digits. It tends to be resistant to both topical and systemic treatments for psoriasis. We present an infant with Acrodermatitis Continua of Hallopeau affecting nineteen nails, with an excellent response to the combination of thalidomide and ultraviolet B phototherapy.

Supresión del tratamiento con zinc en un caso de Acrodermatitis Enteropática / The elimination of the zinc treatment in an Acrodermatitis Enteropathica case

La Acrodermatitis Enteropática es una enfermedad de baja incidencia, se trata de una genodermatosis autonómica recesiva. Sus manifestaciones clínicas son variadas considerándose penta sintomática con lesiones cutáneas, síntomas digestivos, alopecia, distrofia de uñas así como alteraciones psíquicas, fotofobia y depresión mental. En este caso se trata de una paciente, de 29 años de edad, que ha sido tratada en el Servicio de Dermatología Provincial de Matanzas, desde los 6 meses de edad, por presentar lesiones cutáneas alrededor de la boca, vulva y ano, así como en dorso de ambos pies y codos. Esta paciente mediante el cuadro clínico, la biopsia de piel y la dosificación de Zn en sangre y orina se diagnóstico como portadora de Acrodermatitis enteropática congénita, por lo que ha recibido tratamiento con zinc desde esa edad, con recaídas en varias oportunidades, lo que motivó varios ingresos en etapa infantil y además en etapa adulta. En esta oportunidad no les fue posible la adquisición del medicamento, por lo que desde hacía dos meses no realizaba tratamiento, apareciendo lesiones cutáneas como en años anteriores, lo cual motivó el ingreso en nuestro servicio. Se indicó tratamiento con: Sulfato de Zinc remitiendo el cuadro dermatológico una vez iniciado el tratamiento, destacándose que los medicamentos tópicos indicados fueron de escasa efectividad en nuestro caso, por lo que consideramos de gran importancia la presentación de este caso para la práctica médica, dada las variadas manifestaciones clínicas que puede presentar y que sólo pueden remitir con el tratamiento descrito...(AU)

Acrodermatitis enteropathica: case report and review of the literature.

Acrodermatitis enteropathica (AE) is a rare hereditary disorder caused by impaired absorption of zinc from the gastrointestinal tract. It is characterized by acral and periorificial dermatitis, alopecia, and diarrhea. Symptoms usually begin on weaning from breast or formula feeding. We report a full-term, 21-month-old boy with typical skin lesions and decreased plasma zinc level (12 micro g/dl). The patient was given zinc sulfate 40 mg/day and at the end of 1 month his condition had improved significantly. After reviewing the literature we emphasize the important role of zinc in human metabolism and the difference between AE and acquired zinc deficiencies.

Using oral tetracycline and topical betamethasone valerate to treat acrodermatitis continua of hallopeau.

Acrodermatitis continua of Hallopeau (ACH) is a rare type of localized pustular psoriasis. We report the case of a 65-year-old alcoholic woman who had severe inflammatory ACH for 10 years. Initial therapy with sulfasalazine was unsuccessful. The patient was then treated with oral tetracycline and topical betamethasone valerate with occlusive dressing. Her condition improved dramatically after one week.

Acrodermatitis enteropática / Acrodermatitis enterophatica

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

Acrodermatitis enteropática / Acrodermatitis enterophatica

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 (AU)

Acrodermatitis enteropática / Acrodermatitis enteropathica

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

Acrodermatitis enteropathica without hypozincemia: therapeutic effect of a pancreatic enzyme preparation due to a zinc-binding ligand.

The clinical course and intestinal absorption studies of a female infant who developed diarrhea after cessation of breast feeding, mood changes, and intermittently had mild perioral and perianal rashes are described. She showed a partial response to a pancreatic enzyme preparation which was attributed to its content of a zinc-binding ligand, picolinic acid. Complete recovery occurred on pharmacologic doses of zinc. Exacerbation occurred twice upon withdrawal of the oral zinc medication. The zinc concentrations of plasma and intestinal mucosa were normal.

Acrodermatitis chronica atrophicans

Acrodermatitis chronica atrophicans (ACA) is a puzzling, chronic, antibiotic-responsive condition that is rather common in western Europe, bur rarely seen in native Americans.