Brooke-Spiegler syndrome.

The Brooke-Spiegler syndrome is an autosomal dominant one characterized by cylindromas, trichoepitheliomas and occasionally spiradenomas. Within a given family, some members may have cylindromas whereas others may have trichoepitheliomas or both. We describe the coexistence of trichoepithelioma papulosum multiplex (also known as epithelioma adenoides cysticum of Brooke) and cylindromas in a 30-year-old man, and discuss the relationship between these two autosomal dominant syndromes.

[Effectiveness of a micronized purified flavonoid fraction (MPFF) in the healing process of lower limb ulcers. An open multicentre study, controlled and randomized]. / Efficacia della frazione flavonoica purificata micronizzata (FFPM) nell'aumentare la guarigione della ulcere agli arti inferiori. Studio multicentrico in aperto, controllato e randomizato.

OBJECTIVE: To determine the increase in healing rate of venous ulcer in patients receiving a micronised purified flavonoid fraction (MPFF) as supplementation to standard local care. DESIGN: A randomised, open, controlled, multicentre study. SETTING: Departments of Dermatology and University Outpatients Clinics. PATIENTS: One hundred and forty patients with chronic venous insufficiency and venous ulcers. INTERVENTION: PATIENTS received standard compressive therapy plus external treatment alone or 2 tablets of MPFF daily in addition to the above treatment for 24 weeks. MAIN OUTCOME MEASURE: Healing of ulcers and their reduction in size after 24 weeks of treatment. RESULTS: The percentage of patients whose ulcers healed completely was found to be markedly higher in those receiving MPFF in addition to standard external and compressive treatment than in those treated with conventional therapy alone (46.5% vs 27.5%; p<0.05. OR=2.3, 95% CI 1.1-4.6). Ulcers with diameters <3 cm were cured in 71% of patients in the MPFF group and in 50% of patients in the control group, whereas ulcers between 3 and 6 cm in diameter were cured in 60% and 32% of patients (p<0.05), respectively. The mean reduction in ulcer size was also found to be greater in patients treated with MPFF (80%) than in the control group (65%) (p<0.05). The cost-effectiveness ratio (cost per healed ulcer) in the MPFF group was 1026.2 compared with 1871.8 in the control group. CONCLUSIONS: These results indicate that MPFF significantly improves the cure rate in patients with chronic venous insufficiency.

Increased interleukin-7 levels in the sera of psoriatic patients: lack of correlations with interleukin-6 levels and disease intensity.

Interleukin (IL)-7 is a multifunctional cytokine which is involved in the regulation of keratinocyte-T lymphocyte interactions; the latter is an important factor in the pathogenesis of psoriasis. In vitro, IL-7 is able to induce release of cytokines, including IL-6; IL-6 expression is known to be enhanced in psoriatic patients. Serum levels of IL-7 and IL-6 were measured by ELISA in 40 psoriatic patients and compared with cytokine levels in 18 healthy individuals. Serum levels of IL-7 were also evaluated in 24 psoriatic patients during the remission of the disease after effective treatment. The IL-7 and IL-6 serum levels were significantly higher in psoriatic patients than in healthy subjects and the IL-7 serum levels did not significantly decrease after treatment. Serum levels of IL-7 did not correlate with PASI scores; however, a significant positive relationship was observed between IL-6 levels and PASI scores. There was no correlation between increased levels of IL-7 and IL-6 in the sera of psoriatic patients, suggesting the lack of a direct link between these two cytokines in the psoriatic process. In conclusion, increased IL-7 serum levels suggest that IL-7, like IL-6, may be involved in the pathogenesis of psoriasis, but in contrast with IL-6, serum IL-7 levels could not be used as a marker of disease activity in psoriatic patients.

Soluble E-selectin serum levels correlate with disease activity in psoriatic patients.

E-selectin is an adhesion molecule expressed on vascular endothelial cells in several inflammatory skin diseases, including psoriasis. It is responsible for the adherence between microvascular endothelium and neutrophils, monocytes, eosinophils and subsets of T cells. Soluble E-selectin (sE-selectin) serum levels were measured by ELISA in 32 psoriatic patients before treatment and compared with both post-treatment sE-selectin levels in 16 patients and sE-selectin values in 10 healthy individuals. Soluble E-selectin serum levels were significantly increased in psoriatic patients compared with healthy persons. Moreover, a significant correlation was demonstrated between sE-selectin values and PASI scores. No relationship was found between sE-selectin levels and duration of psoriasis. Soluble E-selectin serum levels decreased significantly after treatment of psoriasis. This phenomenon was more evident in patients with more severe psoriasis. In conclusion, sE-selectin serum levels correlate with the extent of psoriatic lesions and could be used as marker of the disease activity in psoriatic patients.

Subcutaneous T-cell lymphoma: report of two cases.

We have seen two patients, a 24-year-old man and a 15-year-old girl, with subcutaneous nodules and fever. The histopathological findings in both cases showed a lobular, histiocytic panniculitis with 'bean bag' cells and lymphoid cells identified as immature T-cells. This picture was consistent with subcutaneous T-cell lymphoma associated with cytophagic histiocytic panniculitis. The girl died after 5 months of disease with florid hemophagocytic syndrome; the man was subjected to a course of treatment (CHOP; cyclophosphamide/doxorubicin/vincristine/prednisone) and after 6 months observation his symptoms of nodules and fever disappeared.

Excess benign melanocytic naevi in renal transplant recipients.

BACKGROUND: Some studies indicate that malignant melanoma occurs more frequently in renal transplant recipients than in the normal population. The development of excess benign melanocytic naevi is regarded as an indicator of the risk for malignant melanoma. OBJECTIVE: This study was undertaken to evaluate the prevalence of benign melanocytic naevi in adult renal transplant patients. METHOD: All benign melanocytic naevi irrespective of size were counted in 76 patients with renal transplants and were compared to naevus counts in 55 sex- and age-matched healthy controls. RESULTS: The mean total number of benign melanocytic naevi was significantly higher (p < 0.001) in renal transplant patients than in the control group: 93.6 +/- 52.2 and 36.1 +/- 29.9, respectively. The most evident increase occurred on the palms/soles and back/buttocks. A positive, although not significant, correlation between naevus counts and duration of immunosuppression was found. CONCLUSION: Renal transplant recipients have an increased number of benign melanocytic naevi and should be considered as a risk group for malignant melanoma.

[Activity of pyruvate kinase and glucose-6-phosphate dehydrogenase in polymorphonuclear granulocytes of patients with atopic dermatitis in the active stage and in remission]. / Aktywnosc kinazy pirogronianowej i dehydrogenazy glukozo-6-fosforanowej w granulocytach wielojadrzastych u chorych z atopowym zapaleniem skóry w okresie zmian czynnych i w okresie remisji.

The activity of granulocyte pyruvate kinase and glucose-6-phospho dehydrogenase in 12 patients with severe atopic dermatitis in active stage of disease and in remission was investigated. It was found that in active stage the activity of both enzymes was not significantly different than in healthy individuals. In remission pyruvate kinase activity was significantly decreased and glucose-6-phospho dehydrogenase activity was found to be non significantly decreased.

[Assessment of the phagocytosis and intracellular killing of Candida albicans by granulocytes of patients with atopic dermatitis]. / Ocena zdolnosci fagocytozy i zabijania sródkomórkowego komórek Candida albicans przez granulocyty chorych na atopowe zapalenie skóry.

Using morphological and radionuclide methods the ability of engulfing and intracellular killing of Candida albicans cells by granulocytes of patients with atopic dermatitis was tested. The patients had moderately intense lesions without secondary infections. The results obtained by the morphological method, that is the percent of phagocytizing granulocytes, the percent of C. albicans cells engulfed by the granulocytes and the percent of killed C. albicans cells in the group of 25 patients and in the group of 25 controls were not statistically significantly different. The radionuclide method with 51Cr labelling of Candida albicans failed to demonstrate differences between 15 patients and 25 controls in the intracellular killing of these cells. No significant effect of the serum of healthy subjects on the functions of the granulocytes and no effect of the serum of the patients on granulocyte functions of controls were noted.

Granulocyte pyruvate kinase in psoriasis vulgaris and psoriasis arthropathica.

The activity of granulocyte pyruvate kinase in 46 patients with psoriasis vulgaris and eight patients with psoriasis arthropathica was investigated. It was found that in both groups of patients the activity of pyruvate kinase was significantly higher than in healthy individuals. This suggests that the rate of glycolysis in granulocytes of patients with psoriasis is markedly increased. The activity of granulocyte pyruvate kinase in 11 psoriatic patients with actively spreading lesions was compared with the activity of this enzyme when the patients were in remission. The activity of the enzyme was significantly increased during the active stage of the disease and returned to normal during remission.

[Zinc content of leukocytes and serum in psoriasis patients]. / Zinkgehalt in Leukozyten und Serum bei Psoriasiskranken.

The mean zinc level in leucocytes from psoriasis patients was found to be decreased by a statistically significant amount, which was not the case in serum. On the basis of data from the literature and our own observations, the role of zinc is discussed in the development of immunologic events.