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Biomedical subjects

P Fabbri

Publications and source records attributed to P Fabbri.

At least 55 records · Page 3Linked to original sources

Dermatitis herpetiformis and vitiligo: report of a case and review of the literature.

We describe the case of a 53-year-old woman presenting papulous and papulovesicular lesions that were highly pruritic, localized mostly in the achromic areas of vitiligo and symmetrically distributed on the elbows, the buttocks, the shoulders and the neck. The histopathological examination performed on the elbow's lesional skin showed the presence of neutrophils and fibrin microabscesses at the tips of dermal papillae, with a few eosinophils, and small separations between the dermis and epidermis just over the infiltrate. The overlying epidermis was uninjured. The performed tests detected IgA anti-endomysium, anti-thyrogloblin, anti-smooth muscle and anti-microsomal fraction autoantibodies; DIF showed the presence of IgA granular deposits at the dermo-epidermal junction, prevalently at the tips of dermal papillae. This is the tenth case reported of an association between dermatitis herpetiformis and vitiligo. Although the two disorders both have immunological pathogeneses, we think that the topographic coexistence of both disorders is coincidental.

Biopsy, Needle↗

A case of vesiculo-bullous Darier's disease associated with bipolar psychiatric disorder.

We describe the case of a 71-year-old men suffering from Darier's disease since his youth and presenting with intensely itching, vesicular and pustular lesions, localized on his arms, legs and on the ulnar side of his right hand. The patient also had a 30 year-history of bipolar psychiatric disorders. The histopathological examination of lesional skin showed suprabasal acantholytic clefting, hyperparakeratosis, and dyskeratosis. Histopathological and immunopathological (direct and indirect immunofluorescence) results, were consistent with vesiculo-bullous Darier's disease. Corticosteroid therapy with methylprednisolone at low dosage (0.2 mg/kg/day) was started with a quick resolution of the vesicular lesions on his arms and legs.

Aged↗

Clinical, histological and immunopathological features of 58 patients with subacute cutaneous lupus erythematosus. A review by the Italian group of immunodermatology.

BACKGROUND: Subacute cutaneous lupus erythematosus (SCLE) is a distinct subset of cutaneous lupus erythematosus clinically characterized by psoriasiform and/or annular lesions and by a mild or absent systemic involvement. OBJECTIVE: The Italian Group of Immunodermatology of the Italian Society of Dermatology and Venereology reviewed the cases of SCLE seen in 10 years (1987-1996). PATIENTS: Forty-six women and 12 men have been retrospectively studied, 42% had annular lesions, 39% psoriasiform ones and 16% both. RESULTS: Lesions were mainly localized on the neck and face and relapsed in spring and autumn. Seventeen patients had 4 or more American College of Rheumatology criteria and could be classified as having systemic lupus erythematosus. The most frequent histopathological alterations were epidermal atrophy, hydropic degeneration of the basal layer and perivascular lymphocytic infiltrate. Deposits of immunoglobulins and C3 at the dermo-epidermal junction on the clinically involved skin were present in 86% of the patients. Dust-like particles in the epidermis were only found in 3% of cases. Anti-Ro/SSA antibodies were found in 71% of the cases and anti-dsDNA only in 5% of cases. CONCLUSIONS: SCLE is a particular subset of cutaneous lupus erythematosus with peculiar clinical and immunopathological features.

Adolescent↗

Soluble CD30 and cyclosporine in severe atopic dermatitis.

BACKGROUND: Allergen-reactive Th2-like cells expressing membrane CD30 are present in the circulation of atopic dermatitis (AD) patients during seasonal allergen exposure. Moreover, CD30+ T cells are present in the lesional skin of AD patients and high levels of soluble CD30 (sCD30) are found in the serum of the same atopic patients. To investigate the immunosuppressive capacity of cyclosporin A (CsA) in AD patients, the sCD30 serum level was determined before and after CsA treatment (5 mg/kg/day) in 10 patients with severe, refractory AD. The sCD30 serum levels before and after CsA therapy together with other serum parameters were correlated with disease activity. METHODS: sCD30 serum levels were detected using a commercial sandwich ELISA; serum eosinophil cationic protein (ECP) levels were determined using a radioimmunoassay (RIA). RESULTS: In all AD patients sCD30 serum levels were increased ranging from 36 to 300 U/ml, with a mean value equal to 135.7 U/ml. After 6 weeks of CsA treatment, not only was there a significant difference between serum sCD30 levels before (mean 135.7) and after (mean 96.2) treatment but even the serum ECP levels before (mean 57.78) and after (mean 18.69) therapy showed an important reduction. Moreover, no significant difference was found between the mean of serum IgE levels before and after treatment, although the values showed a correlation (p = 0.0003). No significant correlations could be demonstrated between sCD30 levels and serum IgE or between sCD30 and ECP serum levels nor between sCD30 levels and blood eosinophil count after CsA treatment. Moreover, a positive correlation (p = 0.001) was instead documented between sCD30 and the severity of the disease. CONCLUSIONS: In this study, CsA therapy results in clinical improvement together with a statistically significant reduction in sCD30 and ECP serum levels in AD patients.

Adolescent↗

The spectrum of cutaneous manifestations in lupus erythematosus--the Italian experience.

We have evaluated the incidence of lupus erythematosus (LE)-specific skin disease in 186 patients with LE, seen retrospectively over a 10-year period at our Dermatology Department and determined the correlation of LE-nonspecific skin disease in patients with systemic involvement. Chronic cutaneous LE (CCLE) with classical discoid lesions (localized, 70%; generalized, 30%) was the most common cutaneous manifestation (72.5%). Subacute cutaneous LE (SCLE) represented only 8% of LE skin disease (annular-polycyclic type, 73%; papulo-squamous type, 27%). Acute cutaneous LE (ACLE) was detected in 15% of our patients: the butterfly erythema was the most frequent skin lesion (96%) while only one case of bullous LE and one case of widespread maculo-papular eruption in association with malar erythema were demonstrated. In 8 patients no LE-specific skin lesions (lupus sine lupo) were found. LE-nonspecific skin lesions were found in 31% of our patients with systemic LE (SLE): Raynaud's phenomenon was found in 23/58 (39.6%), cutaneous small vessel leukocytoclastic vasculitis in 8/58 (13.7%), nonscarring alopecia in 18/58 (31%), lupus pernio in 6/58 (10.3%), hemorrhagic lesions in 4/58 (6.8%), livedo reticularis in 5/58 (8.6%), mucosal ulcers in 3/58 (5.1%) and periungual telangiectasia in 12/58 (20.6%) SLE patients. LE-nonspecific skin lesions are detected only in patients with SLE and usually in the active phases of the disease.

Adult↗

The role of lymphocytes, granulocytes, mast cells and their related cytokines in lesional skin of linear IgA bullous dermatosis.

Linear IgA bullous dermatosis (LAD) is an acquired, heterogeneous, subepidermal blistering disease characterized by linear IgA deposits at the dermoepidermal basement membrane zone (BMZ), often with circulating IgA antibodies to the BMZ. The pathogenetic mechanism, possibly related to the immunophenotype of infiltrating cells, as well as the potential role of cytokines in determining bullous lesions, have not yet been elucidated. An immunohistochemical study was performed with a large panel of monoclonal antibodies [to CD3, CD4, CD8, CD25, CD1a, CD30, CD54, CD50, endothelial leucocyte adhesion molecule-1, vascular cell adhesion molecule-1, myeloperoxidase (MPO), eosinophil cationic protein EG1 and EG2, tryptase, HLA-DR, human interleukin (IL)-3, human IL-5, human IL-8, human IL-4, tumour necrosis factor (TNF)-alpha, interferon (IFN)-gamma and granulocyte/macrophage colony-stimulating factor] using the alkaline phosphatase-antialkaline phosphatase procedure on lesional and perilesional skin of nine patients (one male, eight female; age range 8 months-80 years) with clinical, histological and immunofluorescent proven LAD. The predominant infiltrating cells, distributed mostly inside and below the bullae, were neutrophils and eosinophils which showed intense activation (MPO +, EG1 +, EG2 +). The lymphocytic infiltrate, consisting principally of CD4 +, HLA-DR + and CD30 + T cells, had a predominantly perivascular distribution. Proinflammatory cytokines, such as TNF-alpha and IFN-gamma, showed a moderate focal expression on the dermal perivascular sites; IL-8 was found to have a particularly intense staining on all the epidermal cell layers and at perivascular and vascular sites. Other cytokines, such as IL-4 and IL-5, showed a prevalent intracytoplasmic staining on some cells of the dermal infiltrate (probably mastocytes and lymphocytes), and at the dermal-epidermal separation sites there was also an intense scattered distribution of IL-5. The specific tissue lesions of LAD may be the consequence of the IgA deposits at the BMZ and also of the release of these cytokines together with tissue damage enzymes derived from neutrophils or eosinophils.

Adolescent↗

Consecutive epidemics of Q fever in a residential facility for drug abusers: impact on persons with human immunodeficiency virus infection.

Two large outbreaks of Q fever occurred in 1987 and 1988 in an agricultural community for the rehabilitation of drug users. Approximately 40% of the residents were human immunodeficiency virus (HIV)-positive. Two hundred thirty-five residents presented with clinical evidence of a flulike syndrome that was confirmed to be Q fever; moreover, a large proportion of residents developed an asymptomatic infection. Clinical signs and symptoms were rather nonspecific: fever, malaise, and muscle pain that were often associated with pulmonary symptoms. Single or multiple opacities were detected, with mild interstitial inflammation evident on chest roentgenograms. The source of infection was the sheepfold, which is part of the stock-farming activity of the community. Both outbreaks occurred just after lambing had begun. Residents who were exposed during the first epidemic were protected in the second one. The attack rate among HIV-positive residents was significantly higher than that among HIV-negative residents in the first outbreak, whereas only a slight, marginally significant difference was observed in the second outbreak. The clinical features of Q fever did not differ between HIV-positive and HIV-negative individuals. No cases of relapse or chronic disease were observed.

Adult↗

Ornithine decarboxylase in colonic mucosa from patients with moderate or severe Crohn's disease and ulcerative colitis.

Polyamines, as well as ornithine decarboxylase (ODC), the enzyme involved in their synthesis, were reported to be closely related to cell proliferation. In Crohn's disease and ulcerative colitis, cell destruction and proliferation increase in the active stage. The aim of the present study was to determine the ODC in both involved and uninvolved areas of the colonic mucosa of active Crohn's disease and ulcerative colitis patients. The patients were divided in two groups, owing to the different level of activity (severe or moderate), by means of clinical endoscopy, laboratory, and histology evaluations. Subjects with suspected disease, but endoscopically unconfirmed, were used as controls. In all ulcerative colitis and Crohn's disease patients the ODC values both in involved and uninvolved mucosa were significantly lower than in controls. In severe Crohn's disease ODC was significantly reduced versus moderate Crohn's disease only in affected tissues. In all ulcerative colitis patients (moderate or severe) the ODC was significantly decreased in involved mucosa compared with uninvolved mucosa. Severe ulcerative colitis showed the significantly lowest ODC. We suggest that the significant decrease of ODC in the bowel mucosa is closely related to the severity of the disease. The highest decrease of ODC in ulcerative colitis patients would be due both to the enhanced cell destruction, and to the feed-back from exogenous increased polyamine production (bowel bacteria, cell desquamation). Therefore ODC would be considered a sensitive index of the inflammatory derangement of the mucosa, especially in acute ulcerative colitis. We conclude that this behaviour may result in an enhanced risk of neoplasia.

Adult↗

Hepatitis C virus (HCV) in cryoglobulinaemic leukocytoclastic vasculitis (LCV): could the presence of HCV in skin lesions be related to T CD8+ lymphocytes, HLA-DR and ICAM-1 expression?

An association between mixed cryoglobulinaemia (MC) and hepatotropic viruses, chiefly hepatitis C virus (HCV), has been widely reported. The presence of HCV genomic sequences or HCV-related viral proteins in the serum, purified cryoglobulins, peripheral blood mononuclear cells and into several tissues has suggested an important triggering role for HCV in MC patients. However, only few reports investigated the presence of HCV in cutaneous vasculitis and its potential pathogenetic role. Biopsies of cutaneous purpuric lesions from 5 MC female patients (aged from 40 to 80 years) were carried out for virological and histopathological evaluation. A leukocytoclastic vasculitis pattern was found in 4/5 subjects, while the presence of HCV RNA was detected in 3/5. In only 3 cases biopsy specimens were sufficient for immunohistochemical and direct immunofluorescence (DIF) studies. Immunohistochemical evaluation was performed by means of alkaline phosphatase and monoclonal anti-alkaline phosphatase (APAAP) immune-complexes. In the same skin specimen APAAP and DIF findings were compared with the presence/absence of HCV genomic sequences (PCR technique). In 1 MC patient, the detection of HCV-RNA was associated to a prevalent CD8+ T suppressor pattern with a perivascular and subjunctional distribution as well as an intense expression of second class (HLA-DR) and intercellular adhesion (ICAM-1) molecules on basal keratinocytes, endothelial cells and perivascular infiltrate. These findings suggest a marked inflammatory activation that spreads from endothelial cells to keratinocytes and Langerhans cells. In the 2 HCV-RNA negative specimens the scanty immunopathological staining could indicate a residual activity due to the previous inflammatory event triggered by cryoglobulins. The deposition of circulating HCV-containing immune complexes (CIC) in the skin could be the initial pathogenic event for cryoglobulinemic vasculitis; subsequently CIC could spread from the vascular bed to the perivascular tissue and then could be very rapidly eliminated. If confirmed in larger patients' series these findings could definitely demonstrate a direct role of HCV in the pathogenesis of cryoglobulinemic vasculitis.

Adult↗

The composition of the lupus band test (LBT) on the sun-protected non-lesional (SPNL) skin in patients with cutaneous lupus erythematosus (CLE).

The objective of this study was to analyse the different immunoreactants at the dermo-epidermal junction (DEJ) of patients with cutaneous lupus erythematosus (CLE). Sun-protected non lesional (SPNL) skin biopsies from 65 patients with specific cutaneous manifestations of LE and from 18 patients with other dermatologic diseases were tested using the direct immunofluorescence (DIF) technique. Nineteen out of 65 patients with CLE were affected by systemic LE (SLE). We used the conventional chi-squared test to analyse statistical differences between CLE-SLE and CLE-non-SLE groups in the immunological composition of lupus band test (LBT). C3 was the most common component while IgM were the most frequent immunoglobulins (Igs) of LBT in LE patients. No immunoreactants could be demonstrated at the DEJ in patients with other dermatologic diseases. No statistical differences could be found between CLE-SLE and CLE-non-SLE groups as regards the detection of the different immunoreactants at the DEJ. A positive LBT (even for the presence of only one immunoreactant at the DEJ) performed on SPNL skin represents a useful and specific criterion to distinguish patients with lupus erythematosus (LE) from those without LE. We also believe in a prognostic value of a positive LBT on SPNL skin when the deposition of at least two immunoreactants is demonstrated, and especially if the deposits are composed of IgG.

Antibodies, Antinuclear↗

Th2-like cytokine activity in dermatitis herpetiformis.

Dermatitis herpetiformis (DH) is a chronic subepidermal blistering disease, in which a perivascular cellular infiltrate, composed mainly of CD4+ T lymphocytes together with a varying number of neutrophils and eosinophils, is thought to be important in the pathogenesis of blister formation. The aim of this study was to investigate the potential role of cytokines such as the interleukins IL-4 and IL-5 and to quantify the distribution of T cells as well as their state of activation using alkaline phosphatase-antialkaline phosphatase and reverse transcriptase-polymerase chain reaction (RT-PCR) procedures in seven patients with typical clinical and histological features of DH. A strong extracellular staining with anti-IL-4 monoclonal antibody was detected in the upper dermis with a prevalent perivascular pattern in perilesional areas, whereas in the dermal-epidermal separation sites there was an intense, scattered distribution. IL-5 was intensely expressed, mainly at the intracellular level, by eosinophils and lymphocytes. Concerning RT-PCR, five DH patients showed a strong positive signal for both IL-4 and IL-5 cytokines while two patients showed a faint signal for both IL-4 and IL-5; these last two cases were histologically poor in inflammatory cells. In view of these results, it can be hypothesized that the recruitment of eosinophils and neutrophils in DH may be induced not only by granulocyte macrophage colony-stimulating factor and IL-8 as previously demonstrated, but also by Th2 cytokines as well.

Adolescent↗

In vivo relevance of CD30 in atopic dermatitis.

CD30 expression was evaluated by immunohistochemistry in lesional skin biopsies of eight patients with active atopic dermatitis (AD) and three patients with allergic contact (nickel-induced) dermatitis (ACD). CD30 expression was also assessed in a large panel of CD4+ and CD8+ T-cell clones generated from the skin biopsies of four patients with AD. Finally, the levels of soluble CD30 (sCD30) were measured in the serum of 41 patients with AD, 19 patients with ACD, and 60 healthy controls. In all specimens of lesional AD skin, where the great majority of infiltrating cells were CD4+ T cells, remarkable numbers of cells were CD30+, whereas virtually no CD30+ cells were found in the skin of patients with ACD. In CD4+ T-cell clones generated from the lesional AD skin, most of which produced both interleukin (IL)-4 and interferon-gamma (IFN-gamma) (Th0-like cells) or IL-4 and IL-5, but not IFN-gamma (Th2-like cells), CD30 expression directly correlated with the ability to produce IL-4 and IL-5, but was inversely related to IFN-gamma production. High levels of sCD30 (correlated with disease activity: r = 0.618) were detected in the serum of most AD patients, whereas there was no increase of sCD30 levels in the serum of patients with ACD. These data support the view that Th0/Th2-type responses predominate in the skin of patients with AD and suggest that the presence of CD30+ T cells in tissues and/or increased levels of sCD30 in biologic fluids are indicative of Th2-dominated responses.

Adult↗

Microalbuminuria as a predictor of a drop in glomerular filtration rate in subjects with non-insulin-dependent diabetes mellitus and hypertension.

UNLABELLED: Hypertension and non-insulin-dependent diabetes mellitus (NIDDM) are two major risk factors for end-stage renal failure. The value of microalbuminuria (urinary albumin excretion [UAE]: 30-300 mg/24 h) as an indicator of the glomerular filtration rate (GFR) is not known in these patients. METHODS: The relationships between microalbuminuria and GFR in subjects with NIDDM and hypertension were studied cross-sectionally (Study I) and longitudinally (Study II). RESULTS: In study I, 205 NIDDM subjects with hypertension (151 with normoalbuminuria [UAE < 30 mg/24 h] and 54 with microalbuminuria) were studied. The GFR of subjects with normoalbuminuria (97 +/- 30 ml/min) (mean +/- SD), and microalbuminuria (97 +/- 27 ml/min; NS) were similar. Study II examined 51 of the subjects with normoalbuminuria and 21 with microalbuminuria 22 months (range 13-57) later. The GFR of subjects with microalbuminuria (-10 +/- 19 ml/min) declined more than in those with normoalbuminuria (+4 +/- 17 ml/min; Student's t-test: p = 0.0022). The predictive value of microalbuminuria for a drop in GFR was independent of the antihypertensive treatment used, the follow-up time, or changes in UAE. The only variable linked to GFR loss in subjects with microalbuminuria was an increase in diastolic blood pressure (p = 0.0298). CONCLUSION: Microalbuminuria is a risk factor for a drop in GRF in NIDDM subjects with hypertension, and a reduction in blood pressure is the only effective way to prevent a loss of GFR in subjects with microalbuminuria.

Albuminuria↗