Unilateral milia-type intradermal tophi associated with underlying urate subcutaneous deposition: an uncommon cutaneous presentation of gout.

Tophi develop during the most advanced clinical stage of gout, and are usually located on or around the joints. However, unusual skin features caused by intradermal and/or subcutaneous deposition of tophaceous material at locations other than articular regions have been reported. We present the case of a patient with a condition that has been recently termed 'miliarial gout'. which is only the second such case, to our knowledge. A 51-year-old woman, who had a chronic joint disease that had been diagnosed and treated as psoriatic arthritis, presented with multiple asymptomatic, yellowish-white, firm papules (1-3 mm in size) on erythematous areas on the outside of her left leg. On histological examination of a skin biopsy, uric acid crystals were seen in the dermis and subcutis. The patient also had a raised level of serum urate, consistent with a diagnosis of gout. Treatment with allopurinol led to rapid improvement. Intake of corticosteroids and diuretics was a possible triggering factor for the development of cutaneous tophi in this patient.

Expression of somatostatin receptors in human melanoma cell lines: effect of two different somatostatin analogues, octreotide and SOM230, on cell proliferation.

Somatostatin analogues (SAs) are potential anticancer agents. This study was designed to investigate the expression of somatostatin receptors (SSTRs) in melanoma cells and the effect of two SAs on cell proliferation and viability. Eighteen primary and metastatic human cutaneous melanoma cell lines were treated with octreotide and SOM230. Expression of SSTR1, SSTR2, SSTR3 and SSTR5 was assessed by real-time polymerase chain reaction. Proliferation, viability and cell death were assessed using standard assays. Inhibition was modelled by mixed-effect regression. Melanoma cells expressed one or more SSTR. Both SAs inhibited proliferation of most melanoma cell lines, but inhibition was < 50%. Neither SA affected cell viability or induced cell death. The results suggest that melanoma cell lines express SSTRs. The SAs investigated, under the conditions used in this study, did not, however, significantly inhibit melanoma growth or induce cell death. Novel SAs, combination therapy with SAs and their anti-angiogenic properties should be further investigated.

[Accuracy of pleural fluid cytology in malignant effusions]. / Rentabilidad del estudio citológico del líquido pleural en el derrame maligno.

OBJECTIVE: To assess the usefulness of repeat cytological examination of pleural fluid (PF) for diagnosing malignancy as well as the influence of time length between analyses, effusion's size and pleural fluid biochemistries on the diagnostic yield of cytology. METHODS: Retrospective analysis of 1,427 patients with pleural effusion (PE), including 466 patients with malignant PE. In this latter group, the time length between cytological analysis, the size of the PE, and the biochemical characteristics of PF were recorded. RESULTS: The first cytological analysis had a sensitivity of 48.5%. If this was negative, a second PF specimen was diagnostic in 28.6% of cases, whereas submission of a third PF specimen allowed 10.3% of additional diagnosis. The incidence of positive results depended on the primary tumor (e.g. 66.5% in adenocarcinomas, 30.8% in mesotheliomas), but neither on the time length between cytological analyses nor on the effusion's size. A multivariate analysis showed that a PF to serum glucose ratio

Rentabilidad del estudio citológico del líquido pleural en el derrame maligno / Accuracy of pleural fluid cytology in malignant effusions

Objetivo: Evaluar la rentabilidad de los estudios citológicos sucesivos del líquido pleural (LP) para diagnosticar malignidad y analizar la influencia que sobre aquella tienen el tiempo transcurrido entre los análisis,el tamaño del derrame y las características bioquímicas del LP. Métodos: Se revisaron retrospectivamente 1.427 pacientes con derrame pleural (DP), de los que 466 eran de causa maligna. En este último grupo se analizaron las citologías sucesivas, el tiempo transcurrido entre las mismas, las características bioquímicas del LP y el tamaño del DP. Resultados: La sensibilidad de una primera citología fue del 48,5%. Cuando un primer estudio citológico era negativo, un segundo era diagnóstico en el 28,6% de los casos, mientras que con dos citologías negativas un tercer estudio conseguía un 10,3% de positividades adicionales. El tipo de tumor condiciona la rentabilidad de la citología (66,5% en adenocarcinomas frente a 30,8% en mesoteliomas), pero no así el tiempo transcurrido entre los análisis citológicos sucesivos ni el tamaño del DP. De los parámetros bioquímicos del LP, un análisis multivariante mostró que sólo un cociente entre la glucosa del LP y del suero ≤ 0,75 se relacionaba con una mayor sensibilidad de la citología (74 vs. 47%, p < 0,001). Conclusión: Se aconseja repetir al menos una segunda citología en todo DP de etiología incierta, cuando una primera ha resultado negativa. Este segundo estudio se puede realizar de forma inmediata ya que el paso del tiempo no incrementa la rentabilidad. El porcentaje de positividades está influido por el tipo de tumor y por algunas características bioquímicas del LP, como el cociente entre la glucosa del LP y del suero

Objective: To assess the usefulness of repeat cytological examination of pleural fluid (PF) for diagnosing malignancy as well as the influence of time length between analyses, effusion’s size and pleural fluid biochemistries on the diagnostic yield of cytology. Methods: Retrospective analysis of 1,427 patients with pleural effusion (PE), including 466 patients with malignant PE. In this latter group, the time length between cytological analysis, the size of the PE, and the biochemical characteristics of PF were recorded. Results: The first cytological analysis had a sensitivity of 48.5%. Ifthis was negative, a second PF specimen was diagnostic in 28.6% of cases, whereas submission of a third PF specimen allowed 10.3% of additional diagnosis. The incidence of positive results depended on the primary tumor (e.g. 66.5% in adenocarcinomas, 30.8% in mesotheliomas), but neither on the time length between cytological analyses nor on the effusion’s size. A multivariate analysis showed that a PF to serum glucoseratio ≤ 0.75 was associated with a higher diagnostic yield of cytology (74 vs. 47%, p < 0.001). Conclusion: At least a second PF specimen should be submitted immediately for cytologic analyis in all PE of unknown cause, when the first analysis is not contributory. To delay this second analysis does not increase diagnostic yield. The percentage of cases in which cytologic study of the PF established the diagnosis of malignant PE depends on the tumor type and on certain PF biochemical characteristics such as the PF to serum glucose ratio

Effect of proteasome inhibitors on proliferation and apoptosis of human cutaneous melanoma-derived cell lines.

BACKGROUND: Cutaneous malignant melanoma is an aggressive type of skin cancer which causes disproportionate mortality in young and middle-aged adults. Once disseminated, melanoma can be considered an incurable disease, highly resistant to standard antineoplastic treatment, such as chemotherapy or radiation therapy. The proteasome represents a novel target for cancer therapy that can potentially be used in melanoma. OBJECTIVES: To assess the effect of four structurally different proteasome inhibitors on human cutaneous melanoma-derived cell lines. METHODS: Sixteen human cutaneous melanoma-derived cell lines which are original were obtained from patients who were treated by two of the authors. Cells were cultured, exposed to proteasome inhibitors (bortezomib, ALLN, MG-132 and epoxomicin) and then assayed for cell cycle and cell death analyses. RESULTS: Proteasome inhibitors inhibited the in vitro growth of melanoma cells, and this effect was due to a reduction in cell proliferation rate and an induction of both caspase-dependent and caspase-independent cell death. Moreover, release of apoptosis-inducing factor was observed in the presence of the broad-specificity caspase inhibitor BAF (Boc-D-fmk). In addition, the four different proteasome inhibitors induced caspase 2 processing. CONCLUSIONS: This study provides information regarding the in vitro effects of proteasome inhibitors on melanoma cell lines, and the molecular mechanisms involved. It also gives support to the future use of such inhibitors in the treatment of patients with melanoma, either administered alone or in combination with other drugs.

[Bart syndrome associated to lethal junctional epidermolysis bullosa (Herlitz form)]. / Síndrome de Bart asociado a epidermólisis ampollosa hereditaria letal (Herlitz).

We present the case of a newborn with congenital absence of skin in the anterior part of the left leg that shortly after developed bulla and erosions in hands, feet, ears, buttocks and mouth. The cutaneous biopsy and ultrastructural and immunohistochemical studies showed a subepidermal bulla in the lamina lucida, absence of hemidesmosomes and marked decrease of laminin 5, thus establishing the diagnosis of Bart syndrome associated to the Herlitz form of lethal junctional epidermolysis bullosa. Bart syndrome consists of congenital and localized absence of skin, nail abnormalities and mucoc-cutaneous bullae. It is usually associated to dystrophic epidermolysis bullosa. The Herlitz form of junctional epidermolysis bullosa is a rare variant, usually lethal that is produced by mutations in the genes coding for the anchor protein laminin 5. To our knowledge this is the second case that reports an association between Bart syndrome and lethal junctional epidermolysis bullosa and the first in which the results of immunofluorescence mapping are published.

Síndrome de Bart asociado a epidermólisis ampollosa hereditaria letal (Herlitz) / Bart syndrome associated to lethal junctional epidermolysis bullosa (Herlitz form)

Presentamos el caso de un recién nacido con ausencia congénita de piel en cara anterior de pierna izquierda que poco después desarrolló ampollas y erosiones en manos y pies, pabellones auriculares, nalgas y boca. La biopsia cutánea, el estudio ultraestructural y la inmunohistoquímica mostraron una ampolla subepidérmica en la lámina lúcida, ausencia de hemidesmosomas y marcada reducción de laminina 5, estableciéndose el diagnóstico de síndrome de Bart asociado a epidermólisis ampollosa juncional tipo Herlitz. El síndrome de Bart consiste en ausencia localizada y congénita de piel, alteraciones ungueales y ampollas mucocutáneas. Suele asociarse a epidermólisis ampollosa distrófica dominante. La epidermólisis ampollosa juncional tipo Herlitz es una variante rara, habitualmente mortal, producida por mutaciones en los genes que codifican la proteína de anclaje laminina 5. Que nosotros sepamos, es el segundo caso de asociación entre síndrome de Bart y epidermólisis ampollosa letal y el primero en el que se publican los resultados del mapeo por inmunofluorescencia

We present the case of a newborn with congenital absence of skin in the anterior part of the left leg that shortly after developed bulla and erosions in hands, feet, ears, buttocks and mouth. The cutaneous biopsy and ultrastructural and immunohistochemical studies showed a subepidermal bulla in the lamina lucida, absence of hemidesmosomes and marked decrease of laminin 5, thus establishing the diagnosis of Bart syndrome associated to the Herlitz form of lethal junctional epidermolysis bullosa. Bart syndrome consists of congenital and localized absence of skin, nail abnormalities and mucoc-cutaneous bullae. It is usually associated to dystrophic epidermolysis bullosa. The Herlitz form of junctional epidermolysis bullosa is a rare variant, usually lethal that is produced by mutations in the genes coding for the anchor protein laminin 5. To our knowledge this is the second case that reports an association between Bart syndrome and lethal junctional epidermolysis bullosa and the first in which the results of immunofluorescence mapping are published

Tuberculosis cutánea por Mycobacterium tuberculosis, una patología muy poco frecuente / Cutaneous tuberculosis caused by Mycobacterium tuberculosis, an uncommon pathology

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Corynebacterium ciconiae sp. nov., isolated from the trachea of black storks (Ciconia nigra).

Eight unidentified Gram-positive, rod-shaped organisms were recovered from the tracheas of apparently healthy black storks (Ciconia nigra) and subjected to a polyphasic taxonomic analysis. Based on cellular morphology and biochemical criteria the isolates were tentatively assigned to the genus Corynebacterium, although three of the organisms did not appear to correspond to any recognized species. Comparative 16S rRNA gene sequencing studies demonstrated that all of the isolates were phylogenetically members of the genus Corynebacterium. Five strains were genotypically identified as representing Corynebacterium falsenii, whereas the remaining three strains represented a hitherto unknown subline, associated with a small subcluster of species that includes Corynebacterium mastitidis and its close relatives. On the basis of phenotypic and phylogenetic evidence, it is proposed that the unknown isolates from black storks represent a novel species within the genus Corynebacterium, for which the Corynebacterium ciconiae sp. nov. is proposed. The type strain is CECT 5779(T) (=BS13(T)=CCUG 47525(T)).

Isolation of Corynebacterium falsenii and description of Corynebacterium aquilae sp. nov., from eagles.

Biochemical, molecular chemical and molecular genetic studies were performed on seven unidentified gram-positive, rod-shaped organisms recovered from eagles. The strains were provisionally identified as Corynebacterium jeikeium with the commercial API Coryne system, but they were able to grow under anaerobic conditions and were non-lipophilic. Comparative 16S rRNA gene sequencing studies demonstrated that the isolates belonged phylogenetically to the genus Corynebacterium. Three strains were identified genotypically as Corynebacterium falsenii; the remaining four strains corresponded to a hitherto unknown lineage within the genus Corynebacterium, associated with a small subcluster of species that included Corynebacterium diphtheriae and its close relatives. The unknown bacterial strains were readily distinguished from these and other species of the genus by biochemical tests. Based on both phenotypic and phylogenetic evidence, it is proposed that the unknown bacterial strains from eagles should be classified as Corynebacterium aquilae sp. nov. (type strain is S-613T = CECT 5993T = CCUG 46511T).

Repetitive mucosal trauma promotes colon cancer in experimental rat model.

OBJECTIVE: To investigate the effect of repetitive mucosal trauma, anastomosis and intestinal content on experimental colonic carcinogenesis as there is the possibility than non-specific colon lesions can promote cancer. MATERIAL AND METHOD: We performed to sixty female Sprague-Dawley rats a 4 cm colon loop defunctionalization with double colostomy (traumatic site). Intestinal continuity was restored with an end-to-end colo-colic silk anastomosis. The surviving 47 rats were divided in 3 groups: Group A: 27 rats treated with DMH. Group B: 10 rats treated with EDTA and Group C: Control of 10 rats. Animals were sacrificed 31-32 weeks after surgery for macro and micropathological studies. RESULTS: In group A appeared 60 tumours: 44 in the functional colon, 20 of them in the anastomotic site; 8 in the non traumatised defunctionalized segment and 18 in the traumatised segment (p < 0.05). CONCLUSIONS: a) Continuous microtraumas on colonic mucosa in rats are cancer promotional factors; b) silk suture in anastomosis promotes cancer.

Traumatismos repetitivos sobre la mucosa promueven cáncer / Repetitive mucosal trauma promotes cancer in experimental rat model of colonic carcinogenesis

Objetivo: investigar el efecto de traumatismos repetitivos sobre la mucosa del colon en un modelo de carcinogénesis colónica experimental, ya que es posible que lesiones colónicas inespecíficas sean promotoras de cáncer. Material y método: a 60 ratas hembra Sprague-Dawley les realizamos una desfuncionalización colónica de 4 cm con doble colostomía, restableciendo la continuidad intestinal con sutura término-terminal de seda. Las 47 ratas supervivientes se dividieron aleatoriamente en 3 grupos: Grupo A: 27 ratas tratadas con dimetilhidracina (DMH). Grupo B: 10 ratas tratadas con EDTA y Grupo C: Control de 10 ratas. Se sacrificaron los animales a las 31-32 semanas después de la cirugía para estudio macro y microscópico. Resultados: en el grupo A aparecieron 70 tumores: 44 en colon funcional, 20 de ellos en el lugar de la anastomosis. 26 tumores en el segmento desfuncionalizado, 18 de ellos en su zona traumatizada (p< 0,05). No hubo tumores en los otros grupos. Conclusiones: a) los microtraumatismos continuos en la mucosa del colon son factores promotores de cáncer en la rata; b) la sutura de seda en la anastómosis es un factor promotor de cáncer (AU)

[Histologic study of experimental spleen transplant in rats]. / Studio istologico del trapianto splenico sperimentale nei ratti.

BACKGROUND: The objective of this paper was to demonstrate that the grafts of cervical splenic transplantation on rats using our experimental model present a normal histological appearance. METHODS: Isogenic consanguineous Lewis rats 12 weeks old and weighing 250 gr. were used. Histological findings of a group of 25 cervical splenic grafts transplanted by means of splinting vascular venous microanastomoses and a group of 25 splenic grafts autotransplanted in the omentum were compared with a control group. The specimens were assigned according to a score of 0 to 4, following Moore's histological criteria. RESULTS: All grafts in transplanted and autotransplanted groups had a score of 3 or 4. Then, all splenic grafts from the transplanted group had histological findings very similar to a normal spleen. In the autotransplantation group, the percentage of grafts with a score 3 (60%) was superior to the transplantation group (46%). However, the transplantation group presented a percentage of score 4 (54%), superior to the autotransplantation group (40%). CONCLUSIONS: In our study all grafts from the cervical spleen transplantation group had histological findings very similar to a normal spleen. The percentage of spleens with histological normality in the transplantation group was superior to the autotransplantation group. However, there was no statistical significance.

Grover's disease in patients with chronic renal failure receiving hemodialysis: clinicopathologic review of 4 cases.

In 4 patients undergoing hemodialysis for chronic renal failure, a transient or persistent, papular and keratotic eruption developed on the trunk and arms. Histologic examination disclosed focal acantholysis with dyskeratosis. The lesions were clinically and histologically indistinguishable from those of Grover's disease. A possible association with Grover's disease and chronic renal failure and/or hemodialysis is postulated. Possible implicated pathogenic mechanisms are discussed. We suggest that Grover's disease should be included in the differential diagnosis of cutaneous eruptions in patients with chronic renal failure.

Multiple familial pilomatricomas: a cutaneous marker for Gardner syndrome?

A 40-year-old man and his 6-year-old only son had numerous, firm papulonodular lesions on their faces. Their medical histories were unremarkable and no family consanguinity was recorded. Surgical excision of several lesions was performed on each patient. All the lesions were solid tumors with the characteristic histopathologic features of pilomatricoma. A gastrointestinal radiologic and fibroscopic survey disclosed numerous adenomatous colonic polyps in the father. Additional studies revealed that he also had minor dental abnormalities, a small osteoma on the right mandible, and unilateral, ocular, pigmented retinal macules. The diagnosis of multiple adenomatous colonic polyposis was established only after the well-known association of pilomatricoma-like changes in epidermal cysts in patients with Gardner syndrome was considered. Possibly, multiple familial pilomatricomas could be considered a cutaneous marker of Gardner syndrome.

[Skin metastasis as the lst manifestation of bronchogenic carcinoma]. / Metástasis cutáneas como primera manifestación de carcinoma broncogénico.

Cutaneous metastases as internal neoplasm manifestation are not very frequent. However, the lung cancer in male, is the first cause of cutaneous metastases. The skin metastases may be the first clinical manifestation of cancer in these patients. We report two cases of lung neoplasm in whose first manifestation was metastases of skin. We have reviewed the literature.

[Lymphoepithelioma of the larynx]. / Linfoepitelioma laríngeo.

Non-nasopharyngeal Lymphoepithelioma is a tumor of undifferentiated non-keratinizing cells enclosed in an lymphocytic stroma. The lymphoid tissue does not participate in the tumor process. Its tendency is to precociously spreading in the lymph nodes and viscera, but shows a good radiotherapeutic response. Unlike the nasopharyngeal lymphoepithelioma there is no etiological relationship with the Epstein-Barr virus, in spite of the histological similitudes, therefore the pathogenesis must be different in each. Discrimination between lymphoepithelioma and other neoplasms of the epithelial lineage is important in order to indicate the treatment and also to provide prognostic information.