Coexistence of marginal zone cutaneous B-cell lymphoma and classic Hodgkin disease: does a biological relationship exist?

The coexistence of non-Hodgkin lymphoma (NHL) and Hodgkin disease (HD) in the same patient, although previously reported, is very unusual. This situation is extremely rare when the first diagnosis is a cutaneous B NHL, and exceptional if there is no personal background of cytostatic treatment. We report a 44-year-old man who developed cutaneous nodules over a period of two years. A marginal zone cutaneous B-cell lymphoma was diagnosed. On staging investigation a mass in the lingual tonsil was found and excision biopsy showed a classical Hodgkin lymphoma.

Laboratory paradigms of impulsivity and alcohol dependence: a review.

Impulsivity can be defined as choosing a smaller, immediate reward over a larger, delayed reward. From this perspective, addictive behaviors such as substance abuse and pathological gambling reflect a series of impulsive choices. However, impulsivity is not a homogeneous construct. Laboratory measures of impulsivity reflect two types of processes. The first is related to behavioral inhibition and refers to an individual's ability to appropriately inhibit thoughts or actions. The second is the delay of reward dimension, namely the degree to which immediate (rewarding) consequences have more control over an individual's behavior than consequences that are delayed. In this review, we describe how alcohol is associated with significant impairments in these paradigms. We also suggest that they may have a role in the development of alcohol dependence. These results are in agreement with a model in which delay of gratification might be a marker for early use and/or abuse of alcohol, whereas impairment in behavioral inhibition might be a marker for maintained use in time and, therefore, for progression towards alcohol dependence.

Validation of an Optical Pencil Method to Estimate the Affected Body Surface Area in Psoriasis. / Validación del método de cuantificación del área corporal afectada por la psoriasis mediante lápiz óptico.

INTRODUCTION: Body surface area (BSA) affected by psoriasis is one of the most often used measures for assessing severity, but this method has shortcomings. OBJECTIVE: To validate a new way to estimate BSA. MATERIAL AND METHOD: Prospective, multicenter study in 56 patients with psoriasis. Each patient was evaluated by 2 dermatologists in 2 visits to the same hospital. Each dermatologist used 2 methods for estimating BSA: the traditional visual estimation in which the area of the palm equals 1% of the total body surface and an optical pencil (OP) method in which the affected area is drawn on a touch screen. Software in the application then calculates the BSA. RESULTS: Overall concordance between the 2 methods was acceptable according to an intraclass correlation coefficient (ICC) of 0.87. However, the limits of agreement were unacceptably large and there was systematic bias: traditional estimates were consistently greater than OP calculations. Concordance between the methods was better (ICC>0.8) on the trunk and lower extremities. Intraobserver reliability was excellent with both methods (ICCs, 0.97 and 0.98 for the traditional and OP estimates, respectively). Interobserver reliability was also high (ICCs, 0.91 and 0.94 for the traditional and OP methods), although the mean BSA differed significantly between observers. The ICCs were much lower for BSA estimates on the head. CONCLUSIONS: This study to validate the OP method for estimating the affected BSA in patients with psoriasis shows good agreement between the OP and traditional approaches. The OP calculations also showed less variance and better interobserver reliability.

Stage IV cutaneous acute graft-versus-host disease. Clinical and histological study of 15 cases.

BACKGROUND: The most severe form of cutaneous acute graft-versus-host disease (aGVHD), stage IV, is characterized by the appearance of vesicles and blisters. OBJECTIVE: To describe the clinicopathological characteristics and evolution of stage IV cutaneous aGVHD presented in our hospital. METHOD: Retrospective study. The following criteria for inclusion were applied: (i) patients subjected to allogeneic stem cell transplantation between 1st January 1984 and 31st of December 2006; (ii) development of vesicles and/or blisters; (iii) extracutaneous coincidental aGVHD manifestations; and (iv) presence of histopathological features consistent with aGVHD. RESULTS: Fifteen cases (10 females and 5 males) were studied. The mean age was 38.1 years. The lesions appeared after a median interval of 19 days, always following a milder stage of GVHD. Two patterns of clinical evolution were found. Mucosal involvement was observed in nine patients. Nikolsky's sign was positive in eight patients. Nine of the patients had biopsies of the vesiculobullous stage which showed a subepidermal blister with epidermal necrosis and basal vacuolar degeneration. Only two patients survived. CONCLUSION: Stage IV cutaneous aGVHD is a severe and unusual complication after haematopoietic stem cell transplantation. Prognosis is poor with a very high mortality rate, although the cause of death is varied and not strictly linked to the cutaneous disease.

Psychopathology and Wisconsin Card Sorting Test performance in male schizophrenic patients: influence of dual diagnosis.

BACKGROUND: Different neuropsychological studies have shown schizophrenic patients to have executive function deficits, as illustrated by their performance in neuropsychological tasks such as the Wisconsin Card Sorting Test (WCST); certain studies have described a relationship between these deficits and negative symptoms. Schizophrenic patients also exhibit a high lifetime prevalence (40-50%) of comorbid substance use disorders (SUDs). However, little attention has been paid to this comorbidity (dual diagnosis) in studies associating executive functions and negative symptoms. SAMPLING AND METHODS: Our objective is to investigate the relationship between performance in the WCST and psychopathology as measured by the Positive and Negative Syndrome Scale (PANSS) in a sample of 65 male schizophrenic patients with a history of SUDs (Sch SUD+) and in a sample of 48 male schizophrenic patients without such history (Sch SUD-). RESULTS: In the Sch SUD- group, patients who completed 4 or more categories in the WCST ('good performers') obtained a mean score of 21.2 +/- 8.8 on the negative subscale of the PANSS, compared with a mean score of 27.8 +/- 8.6 in those who completed 3 or less ('poor performers'); these differences were statistically significant (p = 0.015). In the Sch SUD+ group, however, no association was found between WCST performance and the PANSS negative subscale score. CONCLUSIONS: The presence of a history of comorbid SUDs should be taken into consideration in studies investigating executive functions and negative symptoms in schizophrenia.

[Primary cutaneous infections due to Staphylococcus and Streptococcus]. / Infecciones cutáneas primarias por estafilococos y estreptococos.

Pyodermitis constitutes a very important chapter in dermatological practice. Its pathogenesis, in special the one caused by S. aureus, has improved due to the knowledge of the role developed by the host defensins, quorum-sensing signaling and the Panton-Valentine leucocidin gene among others. As well, exfoliative toxins and enterotoxins responsible for the staphylococal scalded skin syndrome (SSSS) and toxic shock syndrome, respectively, have been cloned. Also, it has been seen that bullous impetigo, SSSS and pemphigus foliaceus share a common target: desmoglein-1. The Panton-Valentine leucocidin gene has been related to the appearance of abscesses, necrotic cutaneous lesions and furuncles in young persons caused by meticillin resistant S. aureus, with its epidemiological implications. Finally, some atypical forms of erysipelas suppose a diagnostic challenge for the dermatologist.

Impulsivity and sustained attention in pathological gamblers: influence of childhood ADHD history.

Pathological gambling (PG) has been associated to both impulsiveness and attention deficit/hyperactivity disorder (ADHD) in different studies. Our objective was to compare different impulsivity and sustained attention variables, using both behavioural tasks and self-administered questionnaires, in a group of pathological gamblers with a history of childhood ADHD (PG-ADHD; n = 16), a group of pathological gamblers without this history (PG-non-ADHD; n = 39), and a control group (n = 40). As instruments of measure, we used the stop signal task (to evaluate inhibitory control/impulsivity), the differential reinforcement of Low Rate Responding Task (delay of gratification/impulsivity) and the Continuous Performance Test (sustained attention). The Barratt Impulsivity Scale (BIS-11) was used as a self-administered questionnaire to measure impulsiveness. Our results show that patients in the PG-ADHD group exhibit a significantly lower capacity to delay gratification than those in the PG-non-ADHD and control groups, and less inhibitory control than patients in the PG-non-ADHD group. On self-administered questionnaires such as the BIS-11 the PG-ADHD group obtained higher scores than the PG-non-ADHD and control groups. However, no differences were found with respect to sustained attention using the CPT. Our results suggest a possible selective implication of the prefrontal cortex in PG, which would be especially evident in those with a childhood history of ADHD.

IDRD2 TaqIA polymorphism is associated with urinary homovanillic acid levels in a sample of Spanish male alcoholic patients.

The TaqIA1 allele of the dopamine receptor gene D2 (DRD2) has been associated with alcoholism, as well as with other addictive behaviours. The exact nature of how the presence of this allele can be a vulnerability factor in the development of alcoholism remains unclear. In this study we found that the presence in the DRD2 genotype of the TaqIA1 allele in Spanish alcoholics is associated with higher levels of urine homovanillic acid (HVA) when compared to patients homozygous for the TaqIA2 allele. A sample of 142 Spanish male alcoholic patients was split into 2 groups on the basis of the presence or absence of the A1 allele in their genotype. The urine sample was analyzed by high performance liquid cromatography (HPLC), and the concentration of homovanillic acid (HVA), 5-hydroxyindoleacetic acid (5-HIAA) and vanilylmandelic acid (VMA) was determined. We found a statistical difference in the concentration of HVA between the groups, that suggests this polymorphism could be related to the variance of urine HVA levels.

Water-induced pruritus in haematologically controlled polycythaemia vera: response to phototherapy.

BACKGROUND: Water-induced pruritus is characterized by the development of intense and widespread itching after contact with water at any temperature and without observable skin lesions. Around 40-52% of patients with polycythaemia vera (PV) have water-induced pruritus, and more than 20% of the patients continue with symptoms despite an adequate control of the underlying disease. The aetiology is unknown and treatment is often unsuccessful. We report a patient with a haematologically controlled polycythaemia vera and water-induced pruritus that responded to phototherapy. METHODS: An 83-year-old woman with haematologically controlled PV referred with intense water-induced pruritus without cutaneous lesions. Topical emollients and oral antihistamines were unsatisfactory and so phototherapy treatment (90% UVA/10% UVB) three times a week was commenced. RESULTS: Improvement was visible after 1 month and at the end of 3 months the pruritus had disappeared and treatment was stopped. CONCLUSION: It is considered that the successful treatment in this patient is due to the UVB radiation.

DRD2 and ANKK1 genotype in alcohol-dependent patients with psychopathic traits: association and interaction study.

BACKGROUND: The TaqI-A polymorphism of the ANKK1 gene, adjacent to the DRD2 gene, has been associated with alcoholism and other psychiatric conditions, although other DRD2 gene variants, such as the C957T polymorphism, could be related to these phenotypic traits. AIMS: To investigate the contribution of the TaqI-A and the C957T polymorphisms to the presence of psychopathic traits in patients with alcoholism. METHOD: We performed association and interaction analyses of the polymorphisms in 150 controls and 176 male alcohol-dependent patients assessed for the presence of dissocial personal disorder, using the Psychopathy Checklist-Revised (PCL-R). RESULTS: There was a significant association of the TaqI-A and C957T polymorphisms when both genotypes were present, with PCL-R scores of F(1-171=0.13) (P=0.01) and a frequency of dissocial personal disorder OR=10.52, P<0.001. CONCLUSIONS: The TaqI-A of the ANKK1 gene and the C957T of the DRD2 gene are epistatically associated with psychopathic traits in alcohol-dependent patients.

Unusual clinical presentation of amyloidosis: bilateral stenosis of the external auditory canal, hoarseness and a rapid course of cutaneous lesions.

We report a patient with multiple myeloma associated with primary systemic amyloidosis who had a rapid evolution and a very unusual form of presentation. The association of amyloidosis in patients with multiple myeloma is 15%, and clinically evident mucocutaneous involvement occurs in up to 40% of patients.

Sweet's syndrome on the area of postmastectomy lymphoedema.

BACKGROUND: Sweet's syndrome (SS) has been reported in association with many conditions, including malignancy, infections, autoimmune disorders, pregnancy and drugs. MATERIALS AND METHODS: We reviewed patients with SS-like lesions on the lymphoedema area seen in our department. Clinical manifestations, histopathologic characteristics, treatment and outcome data were recorded and analysed. RESULTS: We report seven women with a history of surgery for breast cancer with axillary lymphadenectomy. Six of them were on tamoxifen. All of them had various lesions consistent with SS localized predominantly on the limb affected by the postmastectomy lymphoedema, and on the ipsilateral chest, trunk and back. One of them presented bullous lesions. Three of the cases underwent spontaneous remission, two resolved with antibiotic therapy, one healed with corticosteroids, and one with corticosteroids plus antibiotic. CONCLUSIONS: Erythematous tender plaques on the area of postmastectomy lymphoedema could be considered an unusual manifestation of Sweet's syndrome. We have found only three similar cases in the literature. Although it is difficult to elucidate the pathogenesis of this entity, it has been suggested that it could be due to immune surveillance impairment.

C957T DRD2 polymorphism is associated with schizophrenia in Spanish patients.

OBJECTIVE: The objective was to confirm whether a homozygous genotype for the C957 allele of the C957T DRD2 gene single nucleotide polymorphism (SNP) is associated with schizophrenia in an independent study population. METHOD: We examined the genotypic distribution of this SNP in a set of clinically ascertained schizophrenic patients (n = 131) and age-matched control subjects (n = 364). Individuals were genotyped using automated analysis of fluorescently labeled PCR products. RESULTS: The distribution of grouped genotypes for the C957T DRD2 SNP (CC vs. CT, TT) showed that C homozygote genotype was over-represented in our patient sample when compared with control subjects. This difference reaches the statistical significance (chi(2) = 7.0; df = 1; P = 0.008; OR = 2.05; % CI 1.2-3.4). CONCLUSION: The findings of this study provide additional evidence that genetic variation at the DRD2 gene plays an important role in the vulnerability to schizophrenia.

Performance in the Wisconsin Card Sorting Test and the C957T polymorphism of the DRD2 gene in healthy volunteers.

INTRODUCTION: Previous studies have associated a decreased striatal D2 dopamine receptor (DRD2) binding with impaired performance in cognitive tasks. In vivo studies have found a lower DRD2 binding associated with the CC genotype of the C957T single nucleotide polymorphism (SNP) of the DRD2 gene. OBJECTIVE: The aim of this study was to investigate the relationship between executive functions and the C957T DRD2 SNP. We hypothesized that the CC genotype would be associated with a poorer executive functioning. METHODS: Our sample consisted of 83 healthy volunteers (28 males and 55 females; mean age 25.2, SD 1.7 years). To assess executive functions, the Wisconsin Card Sorting Test was used, considering the variables perseverative errors, perseverative responses, and number of categories achieved. The genotype distribution was 13 CC, 41 CT, and 29 TT, satisfying Hardy-Weinberg equilibrium. RESULTS: Carriers of the CC genotype, compared with carriers of the CT/TT genotypes, achieved significantly fewer categories (5.00 vs. 5.81; p = 0.004), made a greater number of perseverative errors (13.46 vs. 8.39; p = 0.018), and had a greater number of perseverative responses (14.92 vs. 8.94; p = 0.014). CONCLUSIONS: Our results support the hypothesis that the C957T DRD2 SNP may influence cognitive performance through its repercussions on central dopaminergic function.