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Biomedical subjects

Carlos Ferrándiz

Publications and source records attributed to Carlos Ferrándiz.

17 recordsLinked to original sources

Expression profiles associated with aggressive behavior in Merkel cell carcinoma.

Primary neuroendocrine carcinoma of the skin, or Merkel cell carcinoma, is the most aggressive cutaneous neoplasm. In spite of its similarities to small cell carcinomas from other locations, Merkel cell carcinoma shows many peculiarities probably related to its epidermal origin and the etiologic role of UV radiation. We have immunohistochemically investigated 43 markers on a tissue microarray in which 31 surgically resected Merkel cell carcinomas were represented. Of these, 15 patients remained free of disease after removal, whereas 16 developed metastases. Immunoreactivity was scored according to staining intensity and the percentage of positive cells. We found statistically significant correlations between metastatic tumor spread and overexpression of matrix metalloproteinase (MMP) 7, MMP10/2, tissue inhibitor of metalloproteinase 3, vascular endothelial growth factor (VEGF), P38, stromal NF-kappaB, and synaptophysin. Also detected were statistically significant correlations between the expression levels of MMP7 and VEGF, MMP7 and P21, MMP7 and P38, MMP10/2 and VEGF, P38 and synaptophysin, P38 and P53, and P21 and stromal NF-kappaB. These findings may be helpful in predicting the clinical course of Merkel cell carcinoma and are potentially useful for the development of targeted therapies.

Biomarkers, Tumor↗

Prognostic significance of molecular staging study of sentinel lymph nodes by reverse transcriptase-polymerase chain reaction for tyrosinase in melanoma patients.

BACKGROUND: We performed this study to evaluate the clinical effect of microscopic and submicroscopic metastases in sentinel lymph nodes (SLNs) from patients with early-stage melanoma. METHODS: Patients with confirmed cutaneous melanoma (American Joint Committee on Cancer stages I and II) underwent standard lymphoscintigraphy and SLN biopsy. Serial sections were divided between routine histopathology with hematoxylin and eosin plus immunohistochemistry for HMB-45 and molecular analysis by nested reverse transcriptase-polymerase chain reaction (RT-PCR) assay for tyrosinase (using beta-actin as a control). RESULTS: Of 180 patients analyzed (318 SLNs), 38 (21%) patients had positive SLN(s) by routine hematoxylin and eosin and immunohistochemistry (microscopic disease; group 1), and 142 (79%) had negative histological results. Analysis by RT-PCR detected tyrosinase in at least 1 SLN from 124 (69%) patients. Among patients with histologically negative SLN(s), tyrosinase was detected in 86 (48%) patients (submicroscopic disease; group 2), whereas 40 (22%) patients had negative results by both histology and RT-PCR (group 3). Sixteen (9%) patients had histologically negative SLNs and ambiguous RT-PCR results (group 4). Among 138 patients in the analysis of recurrence (mean follow-up, 45 months), only 18 patients had a recurrence: 11 (31%) of 35 in group 1, 5 (10%) of 51 in group 2, and 2 (5%) of 37 in group 3. No recurrences were seen in group 4. Only group 1 had a significantly shorter disease-free survival and overall survival compared with the other groups. CONCLUSIONS: After a long follow-up period, molecular upstaging by tyrosinase RT-PCR failed to detect a subgroup of patients with an increased probability of recurrence.

Adult↗

Narrow elliptical skin excision and cartilage shaving for treatment of chondrodermatitis nodularis.

BACKGROUND: Surgical excision remains the hallmark of therapy for chondrodermatitis nodularis chronica (CNC). Although excision is generally successful, recurrence at the edge of the excised nodule is well recognized. OBJECTIVE: To analyze the clinical features and the recurrence rate of patients with CNC treated with elliptical excision of the papule and removal of the underlying cartilage. DESIGN: Seventy-four outpatients with CNC, 52 men and 22 women, were retrospectively analyzed. A narrow elliptical excision of the papule followed by a slice of the underlying cartilage were carried out with local anesthetic. Cartilage spikes were trimmed carefully shaping a smooth contour from the normal helical rim to the defect. RESULTS: Good cosmetic results were obtained in all patients. The median follow-up for helical and antihelical lesions were 54 and 50 months, respectively. The global recurrence rate was 13.5% and ranged from 10.6 to 37.5% for helical and antihelical lesions, respectively. CONCLUSIONS: We recommend this surgical procedure because it has been shown to be as efficient as other methods and probably faster to perform.

Adult↗

Clinical spectrum and histological analysis of 32 cases of specific cutaneous sarcoidosis.

BACKGROUND: Sarcoidosis is a multisystem granulomatous disorder of unknown aetiology in which skin involvement is frequent. OBJECTIVE: To review histological characteristics of biopsies of specific cutaneous lesions of sarcoidosis and their relationship with clinical course. PATIENTS AND METHODS: Biopsies from 32 patients with specific cutaneous sarcoidosis were reviewed. Histological findings and clinical characteristics of these patients were analysed. RESULTS: The initial clinical lesions of the patients were ten infiltrated nodule-plaques, eight papules, four maculopapular eruptions, five scar sarcoidosis, four subcutaneous nodules and one lupus pernio. Sarcoidal granulomas were located at dermis in 31 cases (74%) and at subcutaneous fat in 12 (28%) but only four were subcutaneous exclusively. Perivascular or periannexial distribution of granulomas was observed in eight cases (19%) and they had coalescence in 29 samples. The presence of foreign material was demonstrated in 11 cases (26%). CONCLUSIONS: Clinical spectrum of specific lesions of cutaneous sarcoidosis showed a good correlation with granulomas localization in the biopsies. However, traditional classification of specific cutaneous sarcoidosis is often overlapping. On the other hand, foreign bodies and other atypical histological findings were more common than initially expected.

Adult↗

Clinical Experience Acquired with Raptiva (CLEAR) trial in patients with moderate-to-severe plaque psoriasis: results from extended treatment in an international, Phase III, placebo-controlled trial.

BACKGROUND: The 12-week, double-blind, placebo-controlled, first-treatment (FT) CLEAR trial period demonstrated the efficacy/safety of efalizumab in moderate-to-severe plaque psoriasis, including refractory or contraindicated patients unsuitable for other systemic treatments. This study assessed the efficacy/safety of open-label extended treatment (up to 24 weeks' continuous treatment) in patients not achieving > or =75% improvement in Psoriasis Area and Severity Index (PASI-75) at week 12 of the FT period. Time to relapse after treatment cessation, and efficacy/safety of 12 weeks' open-label re-treatment in patients achieving PASI-75 at week 12 FT were also assessed. PATIENTS AND METHODS: Patients with PASI-75 at week 12 FT were observed without treatment until relapse, then re-treated with open-label efalizumab (1.0 mg/kg/week for 12 weeks). Others received open-label extended treatment without intervening observation. RESULTS: Among efalizumab-treated patients (n = 308) who had < 75% PASI improvement at week 12 FT, extended treatment led to PASI-75 in 26.6%. Among patients with between > or = 50 and < 75% PASI improvement at week 12 FT (n = 118), 47.5% improved to PASI-75 with extended treatment. For patients achieving PASI-75 at week 12 FT (n = 164), median time to relapse was 58 days. Re-treatment after relapse led to mean PASI improvement of 62.3% from study baseline (n = 145). Safety results were consistent with previous studies, with no new safety concerns. CONCLUSIONS: These results demonstrate additional benefit of continuing efalizumab. Re-treatment re-established disease control in patients with PASI-75 who relapsed following treatment cessation. The safety profile remained consistent with that seen at 12 weeks.

Administration, Topical↗

[Validation of the Spanish version of the Psoriasis Disability Index questionnaire in assessing the quality of life of patients with moderate-severe psoriasis].

INTRODUCTION: To assess the measurement properties of the Spanish version of the Psoriasis Disability Index (PDI) quality of life questionnaire for patients with moderate or severe psoriasis in ordinary clinical practice. PATIENTS AND METHODS: A study was carried out with 294 patients with moderate or severe psoriasis who had received therapeutic treatment. Sociodemographic and clinical variables were collected and the PDI and EuroQoL-5D questionnaires were administered at the baseline visit, and at 3 and 6 months later. The feasibility (percentage of patients responding to the questionnaire), validity of the construct, internal reliability and sensitivity to change were analyzed. RESULTS: Feasibility: 98.6 % of the patients answered over 80 % of the questions on the questionnaire. Validity of the construct: the PDI scores were correlated with the scores obtained in the Psoriasis Area Disability Index (PASI) (r = 0.33) and the Visual Analogue Scale (VAS) of the EQ-5D (r = -0.41) (p < 0.01). Longitudinal validity: the PDI questionnaire score after 6 months of treatment was correlated with the changes in the PASI index and the VAS (r = 0.39 y -0.51). Reliability: the internal consistency of the dimensions was high (Cronbach's alpha = 0.89). Sensitivity to changes: the size of the effect corresponding to the changes experienced by the patients who noticed an improvement in the severity of their psoriasis during the period between visits was 0.95. CONCLUSION: The Spanish version of the PDI has proven to be reliable, valid and sensitive to changes for use with patients with psoriasis in the Spanish population, although the test-retest reliability should be analyzed for clinically stable patients.

Adult↗

Single-institution experience in the management of patients with clinical stage I and II cutaneous melanoma: results of sentinel lymph node biopsy in 240 cases.

BACKGROUND: Lymphatic mapping and sentinel lymph node biopsy (SLNB) has been developed as a minimally invasive technique to determine the pathologic status of regional lymph nodes in patients without clinically palpable disease and incorporated in the latest version of the American Joint Committee on Cancer (AJCC) staging system for cutaneous melanoma. OBJECTIVE: To analyze the results of SLNB and the prognostic value of the micrometastases and the pattern of early recurrences in patients according to sentinel lymph node (SLN) status. METHOD: Patients with cutaneous melanoma in stages I and II (AJCC 2002) who underwent lymphatic mapping and SLNB from 1997 to 2003 were included in a prospective database for analysis. RESULTS: The rate of identification of the SLN was 100%. Micrometastases to SLN were found in 20.8% of patients. The rate of SLN micrometastases increased according to Breslow thickness and clinical stage. Breslow thickness of 0.99 mm was the optimal cutpoint for predicting the SLNB result. Twenty-four patients (12.3%) developed a locoregional or distant recurrence at a median follow-up of 31 months. Recurrences were more frequent in patients with a positive SLN. Among patients who had a recurrence, those with a positive SLN were more likely to have distant metastases than those with negative SLN. Nodal recurrences were more frequent in patients with a negative SLN compared with those with a positive SLN. CONCLUSIONS: The status of the SLN provides accurate staging for identifying patients who may benefit from further therapy and is the most important prognostic factor of relapse-free survival.

Adult↗

A case of multiple keratoacanthoma centrifugum marginatum.

BACKGROUND: Keratoacanthoma centrifugum marginatum is a rare variety of keratoacanthoma, usually presented and classified as solitary keratoacanthoma. Reported cases of multiple keratoacanthoma centrifugum marginatum are exceptional in the literature. OBSERVATIONS: A 21-year-old man presented for evaluation of erythematous papules and plaques on both legs that had had a peripheral growth over the past year. Clinical and histologic examination showed typical features of keratoacanthoma centrifugum marginatum. Owing to the multiplicity and size of the lesions, treatment with oral isotretinoin was started. It was ineffective. Subsequent treatment with methotrexate combined with oral prednisone led to the regression of all lesions. CONCLUSIONS: The exceptional case of a man with multiple keratoacanthoma centrifugum marginatum is reported. Treatment with methotrexate combined with oral prednisone was effective. This case illustrates how some variants of keratoacanthomas do not really fit into the current classification and how complex the treatment of these tumors is.

Administration, Oral↗

Merkel cell (primary neuroendocrine) carcinoma of the skin with nodal metastasis showing rhabdomyosarcomatous differentiation.

BACKGROUND: We describe a unique case of Merkel cell (primary neuroendocrine) carcinoma of the skin with a lymph node metastasis showing rhabdomyosarcomatous differentiation. Skeletal muscle differentiation has occasionally been described in primary small cell neuroendocrine carcinomas and considered a form of dual differentiation rather than a collision tumor. In the present case, capacity for divergent differentiation appeared late in the course of the tumor, which suggests a clonal origin for both components of the neoplasm. CONCLUSIONS: The coexistence of neural and rhabdomyoblastic types of differentiation, best epitomized by the Triton tumor, has been construed as the product of dual differentiation of cells originated from neural crest-derived ectomesenchyme. Since Merkel cells seem to originate from a pluripotential primitive keratinocyte and not from the neural crest, rhabdomyoblastic differentiation in a metastasis of primary neuroendocrine carcinoma of the skin probably reflects the close proximity between the programs of neural and skeletal muscle differentiation, which would have been sequentially activated in the case we are reporting.

Aged↗

Surgical management of rhinophyma: report of eight patients treated with electrosection.

BACKGROUND: Rhinophyma is the end stage of rosacea, affecting almost exclusively male patients, characterized by a progressive deformity in nasal shape. Depending on the extent of the deformity, rhinophyma may distort the appearance of those affected and patients may become psychosocially disabled. OBJECTIVE: To present the results of electrosurgical technique of sequential delamination with a slightly damped current in rhinophyma. METHODS: Eight healthy male patients affected by moderate to severe rhinophyma were recruited. A radiofrequency electrosurgical unit was used in the cut/coagulate mode at 40 W (3.8 MHz) to remove thin layers of tissue until the nose shape was re-created. RESULTS: All patients achieved acceptable cosmetic results. Cutaneous reepithelialization was achieved at day 14 after surgery. Erythema subsided within 4-8 weeks and healing was complete with minimal scar formation by 8-10 weeks. None of the patients developed hypertrophic scarring and hypopigmentation was minimal. CONCLUSION: Electrosection is as effective as laser treatments and less expensive. Although living in a laser era, electrosurgical remodeling of the nose is a good alternative for the treatment of rhinophyma.

Aged↗

Psoriasis of early and late onset: a clinical and epidemiologic study from Spain.

BACKGROUND: The existence of 2 distinct forms of psoriasis related to age at onset has been postulated. However, precise data regarding the clinical and epidemiologic characteristics of psoriasis depending on the age at onset are still lacking. OBJECTIVE: The purpose of this study was to define the clinical and epidemiologic features of this disease in Spain and to compare patients with psoriasis of early and late onset. METHODS: An observational, analytic, cross-sectional, multicenter study was carried out. From January 1999 to November 1999, 179 participating dermatologists completed a questionnaire detailing the clinical and epidemiologic features of the first 10 consecutive patients with psoriasis seen in their clinical practice. The sample distribution was proportional to the Spanish population. Both statistical and descriptive analyses were performed. RESULTS: Available data were obtained from 1774 patients. The onset of the disease before 30 years of age was significantly associated with a higher incidence of family history of psoriasis, a more severe and extensive cutaneous involvement, and greater psychosocial impact. Guttate psoriasis, nail involvement, evidence of precipitating factors, and a recurrent clinical course were more frequent in this group of patients. Patients with psoriasis of late onset had a less severe clinical course and a more continuous evolution. Palmoplantar pustulosis was more frequent in this group of patients. No significant relationship was detected between the age at onset and development of joint involvement. CONCLUSION: Patients with early and late onset psoriasis often show different clinical and evolutionary features. From the analysis of our data, it seems that 2 different groups of patients with psoriasis related to age at onset can be defined.

Adolescent↗

The ectrodactyly-ectodermal dysplasia-clefting syndrome (EEC): report of five cases.

We report five patients from two different pedigrees with the ectrodactyly-ectodermal dysplasia-clefting syndrome (EEC). All had features of ectodermal dysplasia, but only three had limb ectrodactyly and orofacial clefting. The present patients illustrate the great phenotypic variability in the EEC syndrome. As no single feature, including any of the three cardinal signs, is mandatory for syndrome diagnosis, a meticulous examination of all family members is needed.

Abnormalities, Multiple↗