Carcinoma ductal ecrino: papel de la cirugía de Mohs y revisión de la literatura / Eccrine Carcinoma: The Role of Mohs Micrographic Surgery and a Review of the Literature

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Síndrome de Buschke-Ollendorff / Buschke-Ollendorff syndrome

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Dermatofibroma gigante, una variante infrecuente de dermatofibroma / Giant dermatofibroma, a rare variant of dermatofibroma

El dermatofibroma es una neoplasia cutánea común de crecimiento lento y comportamiento biológico benigno. Existen presentaciones clínicas poco frecuentes como el dermatofibroma atrófico, el dermatofibroma ulcerado y el dermatofibroma gigante que pueden plantear dudas diagnósticas. La extirpación es curativa, aunque en variantes profundas pueden quedar restos que predispongan a la recidiva. Presentamos un nuevo caso de dermatofibromagigante profundo extirpado mediante cirugía micrográfica de Mohs (AU)

Dermatofibroma is a frequent cutaneous neoplasm with slow growth and benign behaviour. There are uncommon clinical variants like atrophic dermatofibroma, ulcerated dermatofibroma and giant dermatofibroma which may cause diagnostic problems. Excision of the tumor is curative, although in deep types the removal can be incomplete, predisposing to recurrence. A new case of deep giant dermatofibroma removed by Mohs micrographicsurgery is reported (AU)

Osteomas múltiples miliares de la cara / Multiple miliary oseoma of the face

Los osteomas miliares múltiples de la cara son una variante infrecuente de osificación cutánea que se caracteriza por la formación de hueso compacto en dermis y/o tejido celular subcutáneo. Se presentan dos casos de osteomas miliares múltiples de la cara afectando a dos pacientes con hiperparatiroidismo secundario a déficit de vitamina D (AU)

Multiple miliary osteomas of the face are a rare variant of osteoma cutis that is characterized by compact bone formation in the dermis and/or subcutaneous tissue. We report two cases of multiple miliary osteomas of the face involving two patients with hyperparathyroidism secondary to vitamin D deficiency (AU)

Photodynamic therapy reduces the histological features of actinic damage and the expression of early oncogenic markers.

BACKGROUND: Photodynamic therapy (PDT) has been shown to be effective in treating nonmelanoma skin cancer (NMSC), especially actinic keratosis (AK). Moreover, there is sufficient evidence of its effectiveness in preventing the appearance of premalignant and malignant lesions in organ transplant recipients. OBJECTIVES: To describe the molecular and genetic changes underlying this preventive effect. METHODS: Twenty-two patients with AK were treated with methyl aminolaevulinate and red light. Biopsies were performed before and 6 weeks after the treatment. Conventional histopathology and immunohistochemistry were carried out. RESULTS: Not only was a reduction in the dysplasia and elastosis observed, but also a decreased expression of Ki-67 and p53. The abnormal findings did not disappear completely in all cases. The expression of cyclin D1 remained stable. CONCLUSIONS: These findings show that PDT has the potential to reduce the histological signs of photoageing. Moreover, the reduction of Ki-67, a marker of proliferation and of p53, a marker of early skin carcinogenesis, indicates a reversal of the carcinogenic process. On the other hand, the fact that one treatment does not clear dysplasia and expression of p53 completely, and the persistence of cyclin D1, indicate that one single treatment, despite showing good clinical results, is not sufficient to clear completely the signs of chronic actinic damage, and thus the risk of NMSC.

Cutaneous endometriosis.

A 32-year old patient presented with an asymptomatic, non-specific tumour located at the site of a previous cesarian scar. The tumor had never bled but its size changed and the color and consistency varied coinciding with the menstrual cycle. The dermatopathologic study showed the existence of ectopic endometrial glandular tissue in the whole thickness of the reticular dermis.

[Lipid nutrition and the epidermal barrier: The connection between immune-mediated inflammatory diseases and peroxisome proliferator-activated receptors, a new therapeutic target in psoriasis and atopic dermatitis]. / Barrera epidérmica y nutrición lipídica. La conexión PPAR e inmunopatología inflamatoria como nuevas dianas de tratamiento en dermatitis atópica y psoriasis.

The authors describe peroxisome proliferator-activated receptor (PPAR) transcription factors as connectors between the enzymatic mechanisms of the epidermal barrier and the abnormal immune and inflammatory responses that characterize atopic dermatitis and psoriasis. Also described is a new connection between lipid metabolism and the epidermal barrier. A suggestion that emerges is that atopic dermatitis and psoriasis share at least 2 pathogenic mechanisms-namely, deficient expression of PPAR-#a and impaired production of interleukin-10 and interferon-γ-in spite of differences in causes and manifestations. A standardized olive oil formulation with powerful bactericidal and fungicidal effects also has the ability to increase serum levels of these 2 cytokines and regulate serum levels of high-density lipoprotein cholesterol in patients at high risk for inflammatory and cardiovascular disease, suggesting that these may be among the mechanisms responsible for the benefits observed following oral and/or topical administration in patients with atopic dermatitis or psoriasis.

Barrera epidérmica y nutrición lipídica. La conexión PPAR e inmunopatología inflamatoria como nuevas dianas de tratamiento en dermatitis atópica y psoriasis / Lipid Nutrition and the Epidermal Barrier: The Connection Between Immune-Mediated Inflammatory Diseases and Peroxisome Proliferator-Activated Receptors, a New Therapeutic Target in Psoriasis and Atopic Dermatitis

Los autores describen los factores peroxisome proliferator-activated receptors (PPAR) como conectores entre los mecanismos enzimáticos de la barrera epidérmica (BE) y las alteraciones inmuno/inflamatorias que caracterizan a dermatitis atópica (DA) y psoriasis. Igualmente, se describe una nueva conexión entre el metabolismo lipídico y la BE. El análisis de estos hechos permite sugerir que DA y psoriasis, aunque diferentes en su causalidad y clínica, exhiben al menos dos hechos patogénicos comunes, que se manifiestan por defectos en la expresión de PPAR-ά y en la producción de IL-10 e IFN-γ. La capacidad de una formulación magistral de aceites de oliva (FMAO) para aumentar los niveles séricos de ambas citocinas, y regular el colesterol HDL sérico, en pacientes con alto riesgo inflamatorio/cardiovascular, junto a sus potentes efectos bactericidas y fungicidas, sugiere que estos sean algunos de los mecanismos responsables de los positivos efectos observados con la FMAO (oral y/o tópica) en pacientes con DA y psoriasis (AU)

The authors describe peroxisome proliferator-activated receptor (PPAR) transcription factors as connectors between the enzymatic mechanisms of the epidermal barrier and the abnormal immune and inflammatory responses that characterize atopic dermatitis and psoriasis. Also described is a new connection between lipid metabolism and the epidermal barrier. A suggestion that emerges is that atopic dermatitis and psoriasis share at least 2 pathogenic mechanisms–namely, deficient expression of PPAR-ά and impaired production of interleukin-10 and interferon- IFNγ–in spite of differences in causes and manifestations. A standardized olive oil formulation with powerful bactericidal and fungicidal effects also has the ability to increase serum levels of these 2 cytokines and regulate serum levels of high-density lipoprotein cholesterol in patients at high risk for inflammatory and cardiovascular disease, suggesting that these may be among the mechanisms responsible for the benefits observed following oral and/or topical administration in patients with atopic dermatitis or psoriasis (AU)

Lipid Nutrition and the Epidermal Barrier: The Connection Between Immune-Mediated Inflammatory Diseases and Peroxisome Proliferator-Activated Receptors, a New Therapeutic Target in Psoriasis and Atopic Dermatitis. / Barrera epidérmica y nutrición lipídica. La conexión PPAR e inmunopatología inflamatoria como nuevas dianas de tratamiento en dermatitis atópica y psoriasis.

The authors describe peroxisome proliferator-activated receptor (PPAR) transcription factors as connectors between the enzymatic mechanisms of the epidermal barrier and the abnormal immune and inflammatory responses that characterize atopic dermatitis and psoriasis. Also described is a new connection between lipid metabolism and the epidermal barrier. A suggestion that emerges is that atopic dermatitis and psoriasis share at least 2 pathogenic mechanisms-namely, deficient expression of PPAR-#a and impaired production of interleukin-10 and interferon-γ-in spite of differences in causes and manifestations. A standardized olive oil formulation with powerful bactericidal and fungicidal effects also has the ability to increase serum levels of these 2 cytokines and regulate serum levels of high-density lipoprotein cholesterol in patients at high risk for inflammatory and cardiovascular disease, suggesting that these may be among the mechanisms responsible for the benefits observed following oral and/or topical administration in patients with atopic dermatitis or psoriasis.

Metástasis cutáneas de presentación atípicade adenocarcinoma de probable origenendometrial / Cutaneous metastases of atypical presentation from adenocarcinoma

Las metástasis cutáneas son poco frecuentes y en ocasiones representa la primera manifestación de una neoplasia desconocida. Aunque la clínica esvariable, generalmente se confunde con un proceso infeccioso o inflamatorio. Las metástasis de localización acral son particularmente raras y el pronósticoes muy malo. Presentamos el caso de un paciente con metástasis en las manos y los pies como signo inicial de una neoplasia no conocida previamente (AU)

Cutaneous metastases are infrequent and in some cases represent the first sign of cancer from an unknown primary site. Although the clinical presentationvaries, they are generally confused with an infectious or inflammatory process. Acral metastases are particularly rare and the prognosis is verypoor. A patient with metastases on the hands and feet as an initial sign of a previously unknown tumour is reported (AU)

Fibroxantoma atípico. Estudio clínicopatológico e inmunohistoquímico de diez casos / Atypical fibroxanthoma. A clinicopathological and immunohistochemical

El fibroxantoma atípico es un tumor infrecuente, de histogénesis incierta. La mayoría de los autores lo consideran una variante superficial del histiocitomafibroso maligno. Generalmente se manifiesta como un nódulo solitario en personas de edad avanzada y zonas expuestas al sol. El diagnósticodel fiborxantoma atípico es siempre de exclusión, y debe diferenciarse mediante inmunohistoquímica de otros tumores fusocelulares como los carcinomas escamosos y melanomas. Describimos las características clínicopatológicas e inmunohistoquímicas de diez casos de fibroxantoma atípico (AU)

Atypical fibroxanthoma is a rare tumor of unknown histogenesis, considered by most authors to represent a superficial or minimally invasive variant ofmalignant fibrous histiocytoma that most often presents as a solitary nodule on sun-exposed skin of the elderly. The diagnosis of atypical fibroxanthomais always one of exclusion. Other spindle-cell tumor such as squamous cell carcinoma and melanoma must be ruled out by immunohistochemical techniques. We report the clinicopathological and immunohistochemical features of ten cases of atypical fibroxanthoma (AU)

Miofibromatosis infantil sistémica en un lactante / Systemic myofibromatosis in an infant

La miofibromatosis infantil se caracteriza por la presencia de nódulos fibrosos, solitarios o multicéntricos, en la piel, músculo, hueso y/o vísceras internas. A pesar de ser una entidad rara y por lo tanto poco conocida por los pediatras, constituye el tumor fibroso más frecuente en la infancia. Las lesiones cutáneas aisladas presentan muy buen pronóstico. Sin embargo, en ciertos casos hay afectación sistémica. En estos casos, puede producir importantes complicaciones e incluso ser causa de muerte, sobre todo en los primeros meses de vida. Estas complicaciones se deben a su naturaleza localmente destructiva, obstrucción de órganos vitales, retraso en el crecimiento o infección. Se presenta el caso de un lactante que en el momento del nacimiento sólo presentaba lesiones cutáneas. Con el tiempo se han hecho evidentes lesiones óseas y en órganos internos. Incluso presentó dificultad respiratoria por parálisis diafragmática que requirió ingreso en la unidad de cuidados intensivos (UCI) pediátrica. Se recomienda el seguimiento estrecho de todos los pacientes con miofibromatosis congénita para evitar o diagnosticar precozmente estas complicaciones (AU)

[Systemic myofibromatosis in an infant]. / Miofibromatosis infantil sistémica en un lactante.

Infantile myofibromatosis is characterized by the presence of solitary or multicentric fibrous nodules in skin, muscle and/or internal organs. Despite being an infrequent entity, and consequently little known by pediatricians, it constitutes the most frequent fibrous tumor in children. Solitary cutaneous lesions have a very good prognosis but in some cases there is systemic involvement. In these cases the disease can produce serious complications and even put the patient's life at risk, especially during the first months of life. These complications are due to the locally invasive nature of the nodules, obstruction of vital organs, growth retardation or infection. We present the case of an infant who at birth presented a skin nodule only. Over time, the infant presented lesions in skin, bone and internal organs. The infant showed respiratory distress requiring mechanical ventilation due to diaphragmatic paralysis. We recommend close follow-up of all patients with infantile myofibromatosis to prevent or make an early diagnosis of these complications

Pilomatrix carcinoma: a clinicopathologic study of six cases and review of the literature.

Pilomatrix carcinoma, the malignant variant of pilomatrixoma, is a rare entity. The authors report on six patients with pilomatrix carcinoma and review the pertinent literature. The lesions showed a predilection for elderly individuals (mean age, 61 years) with a male:female ratio of 5:1, and they presented as dermal or subcutaneous tumors located on the head and neck (5 neoplasms) and chest (1 neoplasm). Tumors varied in size from 0.6 cm to 2.5 cm (mean, 1.78 cm). None of the lesions recurred after wide local excision. On scanning magnification, all tumors showed the architectural features of a malignant neoplasm (asymmetry and poor circumscription, presence of several markedly sized and variably shaped basaloid aggregations, and ulceration). The tumors were composed of pleomorphic basaloid cells with prominent nucleoli and frequent atypical mitoses accompanied by central areas with keratotic material, shadow cells, and foci of necrosis. The tumor nests were surrounded by a desmoplastic stroma and infiltrated the adjacent tissues. Vascular or perineural infiltration was not observed. In one case, the basaloid cells contained abundant melanin pigment in their cytoplasms. Pilomatrix carcinoma is a neoplasm of low-grade malignancy that should be distinguished from the conventional pilomatrixoma and its variants (aggressive pilomatrixoma and proliferating pilomatrixoma), matricoma, and basal cell carcinoma with matrical differentiation. Clinicians and pathologists should be aware of the occurrence of pilomatrix carcinoma because of its potential for distant metastases.

[Leptomeningeal carcinomatosis as presenting symptom of a gallbladder carcinoma]. / Carcinomatosis leptomeníngea como forma de presentación de un adenocarcinoma de vesícula biliar.

INTRODUCTION: Meningeal carcinomatosis is rare accounting for 4-5% in autopsy of patients with solid tumors, and even less frequent, 1%, in its pure form without brain metastases. We report a case of psychosis symptomatic of a meningeal carcinomatosis as presenting manifestation of a gallbladder carcinoma. This clinicopathological combination has not been described previously. CASE REPORT: 74 years-old man. His past medical history included Parkinson's disease treated with L-dopa 50 mg/8 hour and selegiline; duodenal ulceration and hypertiroidism. He started with delirium and visual hallucinations that do not responded to a reduction of L-dopa and suppression of selegiline. The examination of CSF was diagnostic, malignant cells were identified in the initial examination. The patient dead and his autopsy diagnostic was gallbladder carcinoma with meningeal carcinomatosis. CONCLUSIONS: Leptomeningeal carcinomatosis in its pure form is the infiltration of the leptomeninges without brain metastases. It is less than 1% of meningeal metastases from solid tumors. The most frequent primary tumors are: lung, breast, stomach-esophagus, melanoma, colo-rectal, genital and urinary; and the most frequent histological type is adenocarcinoma. The commonest presenting symptoms are focal brain, medullar or radicular symptoms. Psychiatric as isolated symptom are exceptional. Diagnosis is confirmed by the examination of CSF. Malignant cells appear in the first examination in 45-50% of cases. Leptomeningeal carcinomatosis in gallbladder carcinoma is rare; only four cases has been described previously none of them presenting as isolated psychiatric clinical picture.

Carcinomatosis leptomeníngea como forma de presentación de un adenocarcinoma de vesícula biliar / Leptomeningeal carcinomatosis as presentig symptom of a gallbladder carcinoma

Introducción. La meningitis carcinomatosa es una entidad poco frecuente, pues aparece solamente en un 45% de las autopsias de pacientes con tumores sólidos y en un 1% en su forma pura sin metástasis cerebrales. Presentamos un caso de psicosis sintomática de meningitis carcinomatosa pura como manifestación inicial de adenocarcinoma biliar. Esta combinación clinicopatológica no se ha descrito previamente. Caso clínico. Varón de 74 años, con antecedentes de ulcus duodenal e hipertiroidismo, diagnosticado de enfermedad de Parkinson grado 1 en tratamiento con levodopa 50 mg/8 horas y selegilina. El paciente consultó por presentar un cuadro de delirio con alucinaciones visuales que no respondió a la disminución de la dosis de L-dopa y la suspensión de la selegilina. El análisis del líquido cefalorraquídeo (LCR) fue diagnóstico, pues se identificaron células malignas en la primera punción. El paciente falleció y el diagnóstico de autopsia fue de adenocarcinoma de vesícula biliar con leptomeningitis carcinomatosa por dicho tumor. Conclusiones. Se denomina meningitis carcinomatosa pura a la infiltración de la leptomeninge en ausencia de metástasis cerebrales. Aparece en menos del 1% de pacientes con neoplasias sólidas. Los tumores que con más frecuencia producen dicha meningitis son los de pulmón, mama, esófago-estómago, melanoma, colo-rectal, génito-urinario; y la forma histológica más frecuente es el adenocarcinoma. Los síntomas de presentación más frecuentes son los focales cerebrales,medulares o de raíces lumbosacras. Los síntomas psiquiátricos aislados son muy poco habituales. El diagnóstico se confirma por el hallazgo de células malignas en el LCR. La positividad de la primera punción es del 45-50%. La meningitis carcinomatosa por adenocarcinoma biliar es una rareza, sólo descrita previamente en cuatro casos anteriores y en ninguno de ellos se manifestó con síntomas exclusivamente psiquiátricos (AU)

Introduction. Meningeal carcinomatosis is rare accounting for 4-5% in autopsy of patients with solid tumors, and even less frequent, 1%, in its pure form without brain metastases. We report a case of psychosis symptomatic of a meningeal carcinomatosis as presenting manifestation of a gallbladder carcinoma. This clinicopathological combination has not been described previously. Case report. 74 years-old man. His past medical history included Parkinson’s disease treated with L-dopa 50 mg/8 hour and selegiline; duodenal ulceration and hypertiroidism. He started with delirium and visual hallucinations that do not responded to a reduction of L-dopa and suppression of selegiline. The examination of CSF was diagnostic, malignant cells were identified in the initial examination. The patient dead and his autopsy diagnostic was gallbladder carcinoma with meningeal carcinomatosis. Conclusions. Leptomeningeal carcinomatosis in its pure form is the infiltration of the leptomeninges without brain metastases. It is less than 1% of meningeal metastases from solid tumors. The most frequent primary tumors are: lung, breast, stomach-esophagus, melanoma, colo-rectal, genital and urinary; and the most frequent histological type is adenocarcinoma. The commonest presenting symptoms are focal brain, medullar or radicular symptoms. Psychiatric as isolated symptom are exceptional. Diagnosis is confirmed by the examination of CSF. Malignant cells appear in the first examination in 4550% of cases. Leptomeningeal carcinomatosis in gallbladder carcinoma is rare; only four cases has been described previously none of them presenting as isolated psychiatric clinical picture (AU)