Pellagra: a clinical, histopathological, and epidemiological study of 7 cases.

BACKGROUND AND OBJECTIVES: In the developed world, pellagra is a rare condition that is restricted to a small number of at-risk groups. It mainly affects alcoholic patients and those with dietary deficiencies, with intestinal malabsorption, or in treatment with certain drugs. The aim of this study was to analyze the clinical, histopathological, and epidemiological characteristics of patients diagnosed with pellagra in our hospital and to compare the results with the findings traditionally described for this disease. PATIENTS AND METHODS: We undertook a retrospective study of patients with clinical or pathological evidence of pellagra who were seen in our hospital between 1998 and 2009. RESULTS: Seven patients met the inclusion criteria. All were men and the most common predisposing factors were alcoholism and dietary deficiency. All exhibited photosensitivity mainly affecting the forearms and the upper surface of the feet, where the lesions were more severe. The most consistent histopathological findings were the presence of dilated blood vessels with extravasation and little or no inflammatory infiltrate. Various changes were observed in the epidermis, including those suggestive of mild pellagra, such as epidermal pallor and some degree of ballooning of the keratinocytes. Other abnormalities such as epidermal necrosis and hyperkeratosis were also observed. In most patients, pellagra was not initially suspected. Additional noncutaneous findings were observed in almost all cases. CONCLUSIONS: Pellagra should be ruled out in patients with lesions on sun-exposed areas. Predisposing factors for pellagra should be assessed along with the social situation of patients and the presence of digestive or neurological abnormalities.

Pelagra: estudio clínico, histopatológico y epidemiológico de 7 casos / Pellagra: A Clinical, Histopathological, and Epidemiological Study of 7 Cases

Introducción y objetivos: En el mundo desarrollado la pelagra es una entidad rara confinada a unos pocos grupos de riesgo. Afecta especialmente a personas alcohólicas, con transgresiones dietéticas, malabsorción intestinal o en tratamiento con determinados medicamentos. El objetivo del presente trabajo es realizar un estudio de las características clínicas, histopatológicas y epidemiológicas de los pacientes diagnosticados de pelagra en nuestro centro, y compararlo con los hallazgos «clásicos» de esta entidad. Pacientes y métodos: Se realiza un estudio retrospectivo de los pacientes con hallazgos clínicos y/o patológicos de pelagra en nuestro centro en el periodo comprendido entre 1998 y 2009. Resultados: Siete pacientes cumplían los criterios de inclusión. Todos eran varones y los factores predisponentes más importantes fueron el alcoholismo y la transgresión dietética. Todos mostraban un cuadro de fotosensibilidad, donde el dorso de los antebrazos fue el área más afectada y el dorso del pie la zona donde las lesiones eran más graves. Los hallazgos histopatológicos más constantes fueron la presencia de vasos dilatados asociados a una extravasación hemática, con escaso o nulo infiltrado inflamatorio. Los cambios epidérmicos fueron variados e incluyeron cambios sugestivos de pelagra en grado leve, como una palidez de la epidermis y cierto grado de balonización de los queratinocitos, pero también otras alteraciones como ampollas con necrosis epidérmica e hiperqueratosis. En la mayoría de los pacientes la sospecha clínica inicial no fue de pelagra. Casi todos asociaban una discreta clínica extracutánea. Conclusiones: Ante pacientes con lesiones en áreas fotoexpuestas se debe descartar pelagra. Para ello se deben investigar los factores predisponentes de pelagra, la situación social del paciente y la presencia de alteraciones digestivas y/o neurológicas (AU)

Background and objectives: In the developed world, pellagra is a rare condition that is restricted to a small number of at-risk groups. It mainly affects alcoholic patients and those with dietary deficiencies, with intestinal malabsorption, or in treatment with certain drugs. The aim of this study was to analyze the clinical, histopathological, and epidemiological characteristics of patients diagnosed with pellagra in our hospital and to compare the results with the findings traditionally described for this disease. Patients and methods: We undertook a retrospective study of patients with clinical or pathological evidence of pellagra who were seen in our hospital between 1998 and 2009. Results: Seven patients met the inclusion criteria. All were men and the most common predisposing factors were alcoholism and dietary deficiency. All exhibited photosensitivity mainly affecting the forearms and the upper surface of the feet, where the lesions were more severe. The most consistent histopathological findings were the presence of dilated blood vessels with extravasation and little or no inflammatory infiltrate. Various changes were observed in the epidermis, including those suggestive of mild pellagra, such as epidermal pallor and some degree of ballooning of the keratinocytes. Other abnormalities such as epidermal necrosis and hyperkeratosis were also observed. In most patients, pellagra was not initially suspected. Additional noncutaneous findings were observed in almost all cases. Conclusions: Pellagra should be ruled out in patients with lesions on sun-exposed areas. Predisposing factors for pellagra should be assessed along with the social situation of patients and the presence of digestive or neurological abnormalities (AU)

[Pellagra: a clinical, histopathological, and epidemiological study of 7 cases]. / Pelagra: estudio clínico, histopatológico y epidemiológico de 7 casos.

BACKGROUND AND OBJECTIVES: In the developed world, pellagra is a rare condition that is restricted to a small number of at-risk groups. It mainly affects alcoholic patients and those with dietary deficiencies, with intestinal malabsorption, or in treatment with certain drugs. The aim of this study was to analyze the clinical, histopathological, and epidemiological characteristics of patients diagnosed with pellagra in our hospital and to compare the results with the findings traditionally described for this disease. PATIENTS AND METHODS: We undertook a retrospective study of patients with clinical or pathological evidence of pellagra who were seen in our hospital between 1998 and 2009. RESULTS: Seven patients met the inclusion criteria. All were men and the most common predisposing factors were alcoholism and dietary deficiency. All exhibited photosensitivity mainly affecting the forearms and the upper surface of the feet, where the lesions were more severe. The most consistent histopathological findings were the presence of dilated blood vessels with extravasation and little or no inflammatory infiltrate. Various changes were observed in the epidermis, including those suggestive of mild pellagra, such as epidermal pallor and some degree of ballooning of the keratinocytes. Other abnormalities such as epidermal necrosis and hyperkeratosis were also observed. In most patients, pellagra was not initially suspected. Additional noncutaneous findings were observed in almost all cases. CONCLUSIONS: Pellagra should be ruled out in patients with lesions on sun-exposed areas. Predisposing factors for pellagra should be assessed along with the social situation of patients and the presence of digestive or neurological abnormalities.

Estomatitis geográfica / Geographic Stomatitis

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Hemangioma sinusoidal. Estudio inmunohistoquímico con GLUT1 y WT1 / Sinusoidal Hemangioma: Immunohistochemical Analysis with Glucose Transporter 1(GLUT1) and Williams Tumor Protein 1(WT1)

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Medio Michel para inmunofluorescencia / Michel’s transport medium for immunofluorescence

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No disponible

[Based on the incidence of Creutzfeldt-Jakob disease in the Lanzarote healthcare area. Description of two definitive cases]. / A propósito de la incidencia de la enfermedad de Creutzfeldt-Jakob en el área de salud de Lanzarote. Descripción de dos casos definitivos.

We present two cases who have been diagnosed of definitive Creutzfeldt-Jakob disease in the health area of Lanzarote in the period January 2002 to January 2004. The two cases are presented with clinical description, complementary tests -- including electroencephalogram, 14-3-3 protein determination -- study of the prionic protein gene, and histopathologic findings. In this article, we try to show the importance of trying to reach a definitive diagnosis with the histopathologic study once there is clinical suspicion (a diagnosis that is probable or possible). In addition our cases show that communication between the clinical and the epidemiological coordinator of the regional community and the National Center of Epidemiology is very important. We refer to the clear growth in the incidence of the disease in the population of Lanzarote in the period above mentioned. Finally, we discuss whether this growth is or is not an isolated event.

A propósito de la incidencia de la enfermedad de Creutzfeldt-Jakob en el área de salud de Lanzarote: descripción de dos casos definitivos / Based on the incidence of Creutzfeldt-Jakob disease in the Lanzarote healthcare area: description of two definitive cases

Presentamos dos casos de enfermedad de Creutzfeldt-Jakob definitivo que han sido diagnosticados en el área de salud de Lanzarote durante el período de tiempo que comprende desde enero de 2002 a enero de 2004. Los dos casos se presentan con descripción clínica, de pruebas complementarias, incluido electroencefalografía, proteína 14-3-3 en líquido cefalorraquídeo, estudio del gen de la proteína priónica e histopatológico. En este artículo se intenta reflejar la importancia que tiene el hecho de, ante la sospecha clínica (diagnóstico posible y probable), poner los medios adecuados para intentar un diagnóstico definitivo mediante el estudio histopatológico. Se pone de manifiesto también la importancia de la comunicación con el coordinador clínico y epidemiológico de la comunidad autónoma, y de éstos con el Centro Nacional de Epidemiología. Se hace referencia al claro aumento de la incidencia de la enfermedad para la población de dicha área de salud en este período de tiempo. Al final planteamos una reflexión sobre si este aumento es o no un hecho aislado

We present two cases who have been diagnosed of definitive Creutzfeldt-Jakob disease in the health area of Lanzarote in the period January 2002 to January 2004. The two cases are presented with clinical description, complementary tests - including electroencephalogram, 14-3-3 protein determination - study of the prionic protein gene, and histopathologic findings. In this article, we try to show the importance of trying to reach a definitive diagnosis with the histopathologic study once there is clinical suspicion (a diagnosis that is probable or possible). In addition our cases show that communication between the clinical and the epidemiological coordinator of the regional community and the National Center of Epidemiology is very important. We refer to the clear growth in the incidence of the disease in the population of Lanzarote in the period above mentioned. Finally, we discuss whether this growth is or is not an isolated event

Leucemia primaria de células plasmáticas / Primary plasma cell leukemia

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Histogenesis of malignant melanoma of the skin: the role of lymphocytes in the transformation of tissue developmental units.

It is proposed that the completion of both growth and differentiation, in highly specialized progressively evolving multicellular organisms, is achieved throughout two separated but sexually interacting populations of cells (bi-clonal model): 1) the germ line, seated in an obligatory axial position, required for the generation of cytoplasmic replicons, is the source of travelling vectors (spermatozoa, lymphocytes ...) endowed with these potentially transforming nucleoproteins. 2) The centrifugally growing somatic tissues subjected to mitotic senescence. The progressive loss of replicons probably triggers meiotic-like recombinations and differentiation in distal tissues. The shedding of axial replicons to distal meiotic targets seems to be instrumental in transformation, initiating both, embryogenesis and cancer. This theoretical approach is applied to the histogenetic model of malignant melanoma of the skin. In this setting, the axial population is identified as lymphocytes involved in a process of extrathymic maturation (lichenoid lesions), in the same field where melanoma would arise. Pagetoid nests of melanocytes are considered to be the expression of TDUs evolving in a distal population of senescent neurocristic cells. This situation establishes the likelihood of mixing between potentially transforming lymphocytes and the meiotic melanocytes resulting in a transformed hybrid, the earliest cellular expression of malignant melanoma.

Is a bi-clonal interaction, analogous to that underlying embryogenesis the origin of the earliest malignant phenotype?

We propose a theoretical model of carcinogenesis which parallels embryonic induction. It is based on the interaction between two clones of cells. One of them is of germ cell ancestry and is the source of transforming effector cells. The second clone is the potential target of such travelling "lymphocytoid" cells and emerges in tissues exhibiting the precancerous picture of dysplasia. In atypical fields each endopolyploid cell, arrested in G2 and involved in genomic changes, is the stem of a clonal growth. This emerging new phenotype is subjected to policing control by wandering effector cells. This situation establishes the probability of a random collision between two cells pertaining to different lineages, with two possible antithetical results, cytolysis or cell fusion comparable to fertilization. The latter event is suggested to determine malignancy.