Objectives To investigate clinical, serologic, histopathologic and immunopathologic markers of PURPLE (painful purpuric ulcers with reticular pattern of lower extremities) or atrophie blanche.
Design ...Twelve consecutive patients affected with idiopathic PURPLE were studied in the period 1992–1996. Patients with systemic diseases correlated to PURPLE were excluded from the study.
Subjects All twelve patients were hospitalized at the Istituto Dermopatico Immacolata in Rome.
Results Various and dishomogeneous immunological alterations were detected. Circulating immune complexes, increased serum levels of anticardiolipin and antinuclear antibodies, reduced serum complement levels, and deposition of immunoreactants in dermal vessels, were found in a limited number of patients. The results were not statistically significant. All the patients showed very similar histopathological aspects, characterized by microvascular thromboses, endothelial swelling and segmental hyalinization of small dermal vessels.
Conclusion PURPLE is a thrombogenic vasculopathy in which unspecified immunological and laboratory changes are present. The recently suggested pathogenetic role of anticardiolipin and protein C deficiency in the disorder is not confirmed by this study.
To assess the role of platelets and lymphocyte-related immunological mechanisms in livedo vasculopathy (LV) and cutaneous small vessel vasculitis (CSVV). Livedo vasculopathy is thought to be related ...to the thrombotic occlusion of small and medium-sized dermal vessels. Cutaneous small vessel vasculitis comprises a heterogeneous group of disorders in which the main pathogenetic events could be modulated by circulating cytokines.
Case series study of 2 groups of patients affected respectively with LV and CSVV.
A large clinical and research institute for the study and treatment of cutaneous diseases.
Consecutive patients with clinically and histologically proved idiopathic LV (n = 8) and CSVV (n = 20) were studied and compared with healthy donors (n = 20). Patients with potentially correlated systemic diseases were excluded.
Surface expression of platelet P-selectin and circulating level of interleukin (IL) 1beta, tumor necrosis factor alpha (TNF-alpha), IL-8, IL-2, and soluble IL-2 receptor.
The IL-2 and soluble IL-2 receptor levels were significantly higher in serum samples from patients with both LV (1.24 +/- 0.46 IU/mL mean +/- SD vs 0.46 +/- 0.24 IU/mL, P<.001; 899 +/- 368 IU/mL vs 628 +/- 132 IU/mL, P<.02) and CSVV (0.91 +/- 0.57 IU/mL, P<.02; 1087 +/- 451 IU/mL, P<.001) than in those from the healthy controls. The serum levels of IL-1beta, TNF-alpha, and IL-8 were higher in patients with CSVV than in controls (7.53 +/- 6.7 pg/mL vs 4.58 +/- 2.72 pg/mL; 23.7 +/- 12.6 pg/mL vs 10.82 +/- 2.46 pg/mL, P<.001; 37.8 +/- 46 pg/mL vs 8.25 +/- 3.53 pg/mL, P<.02, respectively). No significant difference in the serum levels of IL-1beta (7.2 +/- 4.9 pg/mL), TNF-alpha (12.9 +/- 3.47 pg/mL), and IL-8 (5.9 +/- 4.13 pg/mL) was observed in patients with LV compared with controls. An increased expression of platelet P-selectin was also detected in patients with LV in comparison with controls and patients with CSVV. The mean +/- SD percentage of positive cells for P-selectin was 43% +/- 5% in the patients with LV, 5.1% +/- 2% in the controls (P<.001), and 5.3% +/- 2% in the patients with CSVV (P<.001).
Taken together, these data demonstrate that different pathogenetic mechanisms are operative in LV and CSVV. In fact, platelet and lymphocyte activation is present in LV, whereas the levels of inflammatory mediators are in a normal range. In CSVV, the high serum levels of proinflammatory cytokines suggest that they are actively involved in the pathogenesis of cutaneous vasculitis.
La diagnostica clinica in dermatologia si basa sul colore della manifestazione cutanea, sulla distribuzione lineare e topografica delle lesioni e sull’interpretazione morfologica delle figure che ...tali lesioni tendono a comporre. L’elaborazione della diagnosi di una malattia cutanea si fonda principalmente su un processo di analisi visiva che involontariamente un dermatologo compie a ogni visita e si traduce in una diagnosi clinica che spesso non ha bisogno di conferma con esami laboratoristici e/o strumentali.
Pemphigus foliaceus treated with etanercept Gubinelli, Emanuela; Bergamo, Fabio; Didona, Biagio ...
Journal of the American Academy of Dermatology,
12/2006, Volume:
55, Issue:
6
Report
Patients affected by some genetic skin defects, for example, dyskeratosis congenita or scleroderma, may present spontaneous or induced chromosomal fragility. Hence we performed a cytogenetic analysis ...in families of patients affected by lamellar ichthyosis, an autosomal recessive disease not yet fully characterized at the cellular and molecular levels. Chromosomal fragility was assayed in untreated lymphocyte cultures and in those supplemented with aphidicolin or bleomycin. Cells from some affected patients and some of their parents showed hypersensitivity to the radiomimetic agent bleomycin.
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