Manifestaciones dermatológicas de fibrosis quística en un lactante: acrodermatitis enteropática símil y pelo gris / Cutaneous manifestations of cystic fibrosis in an infant: acrodermatitis enteropathica and gray hair

Introducción: Las manifestaciones dermatológicas en el momento del diagnóstico de fibrosis quística son infrecuentes. Objetivo: Describir el caso de una lactante con una presentación dermatológica no habitual de fibrosis quística. Caso Clínico: Lactante que a los 2 meses de edad presenta un mal incremento pondoestatural. A los 4 meses presenta exantema cutáneo, edema y pelo gris. Dentro de su estudio se evidencia hipoproteinemia y anemia, y se confirma el diagnóstico de fibrosis quística mediante el estudio genético. Las alteraciones dermatológicas se revirtieron completamente tras la instauración de la terapia de reemplazo con enzimas pancreáticas. Este es el segundo caso reportado de pelo gris en niños con esta enfermedad. Conclusión: En niños con malnutrición que desarrollan alteraciones de la piel y fanéreos debemos sospechar enfermedades metabólicas como fibrosis quística.

Introduction: Cutaneous manifestations at the time of CF diagnosis are rare. Objective: To describe the case of an infant with an unusual cutaneous presentation of cystic fibrosis. Case report: The case is presented of an infant with delayed physical growth at two months, and at the age of four months, presented with a skin rash and gray hair. Tests revealed the presence of hypoproteinemia and anemia, with the diagnosis of Cystic Fibrosis being confirmed by genetic testing. The rash was completely resolved after pancreatic enzyme replacement therapy. This is the second gray hair case reported in children with this disease. Conclusion Metabolic diseases such as cystic fibrosis should be suspected in malnourished children who develop skin disorders.

Acrodermatitis due to zinc deficiency after combined vertical gastroplasty with jejunoileal bypass: case report / Acrodermatite por deficiência de zinco após associação de gastroplastia vertical com derivação jejuno-ileal: relato de caso

CONTEXT: Nutritional complications may occur after bariatric surgery, due to restriction of food intake and impaired digestion or absorption of nutrients. CASE REPORT: After undergoing vertical gastroplasty and jejunoileal bypass, a female patient presented marked weight loss and protein deficiency. Seven months after the bariatric surgery, she presented dermatological features compatible with acrodermatitis enteropathica, as seen from the plasma zinc levels, which were below the reference values (34.4 mg%). The skin lesions improved significantly after 1,000 mg/day of zinc sulfate supplementation for one week. CONCLUSIONS: The patient's evolution shows that the multidisciplinary team involved in surgical treatment of obesity should take nutritional deficiencies into consideration in the differential diagnosis of skin diseases, in order to institute early treatment.

CONTEXTO: Complicações nutricionais podem ocorrer após cirurgia bariátrica, pela restrição no consumo de alimentos e por comprometimento do processo digestivo e absortivo. RELATO DO CASO: Após ter sido submetida a gastroplastia vertical e derivação jejuno-ileal, uma paciente apresentou acentuada perda de peso e desnutrição proteica. Sete meses após a cirurgia bariátrica, manifestou-se quadro dermatológico compatível com acrodermatite enteropática, verificado a partir dos níveis plasmáticos de zinco (34,4 mg%), que se situavam abaixo dos valores de referência. As lesões cutâneas melhoraram significativamente após 1.000 mg/dia de suplementação de sulfato de zinco, por uma semana. CONCLUSÕES: A evolução da paciente mostra que a equipe multiprofissional envolvida no tratamento cirúrgico da obesidade deve considerar as deficiências nutricionais no diagnóstico diferencial das doenças cutâneas, a fim de instituir precocemente o tratamento.

Síndrome de Gianotti-Crosti: reporte de dos casos y revisión de la literatura / Gianotti-Crosti syndrome: report of 2 cases and literature review

El síndrome de Gianotti-Crosti, también llamado acrodermatitis papular de la infancia, es un exantema infrecuente de la infancia, que se presenta generalmente en niños entre 1 y 6 años de edad. Dado que estos pacientes generalmente son llevados a su pediatra, es fundamental el conocimiento de las características de este síndrome. El cuadro clínico se caracteriza por una reacción pápulo-vesicular asintomática y autolimitada de distribución simétrica en cara, nalgas y superficies extensoras de las extremidades, formando placas o permaneciendo aisladas. El tronco generalmente se encuentra respetado y las lesiones usualmente no son pruriginosas. Se reportan los casos de dos pacientes que consultaron en el Servicio de Dermatología de la Pontificia Universidad Católica de Chile, por un cuadro compatible con síndrome de Gianotti-Crosti. A propósito de los casos, se presentan los aspectos más relevantes sobre esta patología.

The Gianotti-Crosti syndrome, also known as papular acrodermatitis of childhood, is an uncommon rash that usually occurs in children between 1 and 6 years old. Given that these patients are usually brought to their pediatrician, it is essential to know the characteristics of this syndrome. The clinical picture is characterized by a self-limited and asymptomatic papulo-vesicular reaction, symmetrically distributed on the face, buttocks and extensor surfaces of the extremities. The trunk is generally respected and the lesions are usually not itchy. We report the cases of two patients treated at the Dermatology Department at the Pontificia Universidad Católica de Chile, with symptoms consistent with Gianotti-Crosti syndrome. We also present the most relevant aspects of this disease.

Acroangiodermatite (pseudossarcoma de Kaposi): uma condição raramente reconhecida. Um caso na planta do pé associado a insuficiência venosa crônica / Acroangiodermatitis (pseudo-Kaposi sarcoma): a rarely-recognized condition. A case on the plantar aspect of the foot associated with chronic venous insufficiency

A acroangiodermatite ou pseudossarcoma de Kaposi é entidade angioproliferativa incomum relacionada a insuficiência venosa crônica, fístulas arteriovenosas, membros paralisados, cotos de amputação, síndromes vasculares e condições trombóticas. Apresenta-se, em geral, como máculas, pápulas ou placas purpúricas no dorso dos pés (especialmente hálux) e maléolos. Relatamos um caso de acroangiodermatite afetando a região plantar, por dois anos sem diagnóstico, para o qual a coloração histológica por hematoxilina-eosina e a marcação imuno-histoquímica com CD34 foram decisivas. A paciente tinha insuficiência venosa crônica e a lesão respondeu bem ao uso de bandagens elásticas e repouso com a perna elevada.

Acroangiodermatitis, often known as pseudo-Kaposi sarcoma, is an uncommon angioproliferative entity related to chronic venous insufficiency, arteriovenous fistulae, paralysed limbs, amputation stumps, vascular syndromes and conditions associated with thrombosis. It presents most frequently as purple macules, papules or plaques in the dorsal aspects of the feet, especially the toes, and the malleoli. We report a case of acroangiodermatitis in the plantar aspect of the foot, misdiagnosed for two years, in which haematoxylin-eosin hystopathological stain and immunolabeling with CD34 histochemistry examination were decisive for diagnosis. Patient had chronic venous insufficiency. The lesion responded well to the treatment with a combination of leg elevation and compression.

Periorificial and acral dermatitis in a newborn having milk intolerence.

A 2-week-old infant born at 36-week gestation developed diarrhea and metabolic acidosis when he was put on formula feeding. He was treated for sepsis and was screened for metabolic diseases. Blood and cerebrospinal fluid cultures were clear. The diarrhea and metabolic acidosis settled but recurred when formula feeding was resumed. He developed a florid erythematous rash involving the palms, feet, perioral and perineal regions. Zinc deficiency was confirmed and zinc replacement resulted in prompt resolution of the skin rash. The patient was put on Pepti-Junior and remained well. This case illustrates that zinc deficiency must be sought and treated in an infant with a typical rash involving the palms, feet and body orifices.

Propionic acidemia and zinc deficiency presenting as necrolytic migratory erythema

Necrolytic migratory erythema was first described in 1942 in a patient with pancreatic islet cell carcinoma. The disease can, however, have other etiologies including nutritional dermatoses. Here, we describe the clinicopathological picture of a 7-year-old female patient who presented with necrolytic migratory erythema which we believe is secondary to a rare combination of zinc deficiency and propionic acidemia

Gianotti-Crosti Syndrome Following Japanese Encephalitis Vaccination

We report a three-year-old Korean boy who presented with itching symmetrical erythematous macules and papules on his face, trunk, and extremities for 1 week. Lymphadenopathies were detected on physical examination. He was vaccinated against Japanese B Encephalitis (JE) 1 day before developing skin rashes. The patient's serum JE antibody titer by hemagglutinin inhibition (HI) test was 1:40. Under the diagnosis of Gianotti-Crosti syndrome following JE vaccination, he was conservatively treated with an antihistamine agent, and his symptoms were all cleared 2 weeks after treatment.

A Case of Chronic Pancreatitis Associated with Liver Infarction and Acrodermatitis Enteropathica

Liver infarction and acrodermatitis enteropathica are rare complications of chronic pancreatitis. This report shows the case of a 56-year-old man who developed liver infarction due to portal vein thrombosis from chronic pancreatitis and acrodermatitis enteropathica during the course of his treatment. The rare combination of these complications in a patient with chronic pancreatitis has never previously been reported in the literature.

Acroangiodermatitis: informe de un caso y revisión de la literatura / Acroangiodermatitis: inform of a case and review of the literature

La acroangiodermatitis es un proceso vascular benigno localizado en las extremidades inferiores, el cual clínica e histológicamente puede semejar a un Sarcoma de Kaposi. Clínicamente se caracteriza por máculas o placas violáceas a marrones en las superficies dorsales de los pies. Se han encontrado varias condiciones asociadas a esta entidad las cuales frecuentemente son mal diagnósticadas y tratadas, de allí la importancia de diferenciarla de un Sarcoma de Kaposi. Se presenta un caso en una paciente de 60 años de edad, quien presentó una lesión eritematoviolácea en el primer dedo del pie izquierdo. Realizamos revisión de la literatura

Borreliosis: la enfermedad de Lyme y otras dermatosis posiblemente asociadas / Borreliosis: Lyme's disease and other possibly associated diseases

Objetivo: actualizar el rol de las borrelias, en relación con varios cuadros nosológicos de interés dermatológico de etiología oscura, que han sido estudiados recientemente y deben ser más conocidos por los dermatólogos prácticos. Se analiza la enfermedad de Lyme y se discuten brevemente la esclerodermia localizada, liquen escleroso y atrófico, fasceítis eosinofílica, atrofodermia de Pasini y Pierini, granuloma anular, paniculitis septal, nódulos fibrosos subcutáneos, enfermedad de Jessner-Kanof, la hemiotrofia facial de Parry-Romberg y el linfoma B cutáneo primario que parecen asociarse con borrelias. La enfermedad de Lyme es un cuadro infeccioso sistémico, causado por espiroquetas del género Borrelia, transmitidas al hombre por garrapatas de género Ixodes. Tiene diversas manifestaciones cutáneas a lo largo de cada una de las etapas por las que atraviesa el proceso, por ejemplo el eritema migrans y el linfocitoma cutis (LC) en el estadío temprano y la acrodermatitis crónica atrofiante (ACA) en el estadío tardío. Se acompaña de síntomas principalmente neurológicos y reumatológicos. Existe tratamiento antibiótico útil, con dixiciclina, amoxicilina o eritromicina. Respecto a las otras enfermedades enumeradas, la terapia antibiótica anti-borrelia es todavía de valor discutible. Conclusión: si las diferentes entidades asociadas con borrelias son marcadores cutáneos de la enfermedad de Lyme o simplemente hallazgos coincidentes, es algo que aún está por determinarse

Síndrome de Gianotti-Crosti: informe de 18 casos / Gianotti-Crosti syndrome: report of 18 cases

En el presente estudio se presentan 18 casos de síndrome de Gianotti-Crosti, que se documentaron de marzo de 1987 a noviembre de 1994, en el Hospital Regional No. 1 de Acapulco, y en el hospital General de Zona No. 14 de guadalajara del Instituto Mexicano del Seguro Social (IMSS). Se describen algunas características epidemiológicas, las manifestaciones clínicas y morfológicas y los hallazgos histipatológicos de laboratorio de la enfermedad

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

Acrodermatite enteropática: a propósito de um caso / Acrodermatitis enteropathica: apropos of a case

Apresentamos um caso típico de Acrodermatite Enteropática com lesöes exuberantes e periorificiais, fotofobia, esteatorréia, alopécia e adinamia. Recordamos a importância do zinco no tegumento cutâneo, seu modo de absorçäo e os meios que levam a uma carência deste oligoelemento