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Biomedical subjects

P Amerio

Publications and source records attributed to P Amerio.

At least 19 recordsLinked to original sources

Vitiligo associated with other autoimmune diseases: polyglandular autoimmune syndrome types 3B+C and 4.

Vitiligo is a common skin disease characterized by depigmented maculae resulting from a reduction of the number and function of melanocytes. Many studies suggest that vitiligo might be an autoimmune disease. Vitiligo has been frequently described in association with other autoimmune diseases. Among the diseases described in association with vitiligo are the so-called autoimmune polyglandular syndromes (APS). Vitiligo can be present in all types of APS but the most frequent association appears to be in APS-3. APS-3 was defined as the association between autoimmune thyroiditis and another autoimmune disease. Here we report one patient with thyroiditis, vitiligo and autoimmune gastritis (APS-3B+C), one patient with chronic autoimmune thyroiditis, vitiligo and alopecia (APS-3C), and one case of a young patient with type 1 diabetes mellitus and vitiligo (APS-4), according to the newest classification. We stress the importance of a thorough assessment for autoimmune diseases in selected patients with vitiligo.

Adult↗

Primary cutaneous CD30+ T-cell lymphoma responsive to topical imiquimod (Aldara).

CD30+ anaplastic large cell lymphoma is a primary cutaneous lymphoproliferative disorder with a high rate of spontaneous regression (almost 25%). The suggested therapies are radiation, surgery and methotrexate. We describe two patients with nonregressing primary cutaneous CD30+ T-cell lymphoma that was successfully treated with topical imiquimod 5% cream (Aldara, 3M) three times weekly for 6 weeks. In both cases we obtained complete clinical remission, confirmed by histology. No recurrences were observed during the following 8 months. We consider that topical application of an immune response modifier, such as imiquimod, could be a good alternative to other potentially more dangerous or aggressive treatments.

Administration, Topical↗

Skin tolerability and efficacy of combination therapy with hydrogen peroxide stabilized cream and adapalene gel in comparison with benzoyl peroxide cream and adapalene gel in common acne. A randomized, investigator-masked, controlled trial.

BACKGROUND: Combination therapy with antiseptics such as benzoyl peroxide (BP) and topical retinoids is widely used as first-line treatment for acne vulgaris (AV). However, these combinations could have a suboptimal skin tolerability. Recently, a new formulation of hydrogen peroxide (HP) 1% in stabilized cream (Crystacide; Mipharm, Milan, Italy) became available. A previous clinical study has shown that HP cream monotherapy presents a better skin tolerability in comparison with BP in patients with mild AV. OBJECTIVES: To evaluate the tolerability and the efficacy of combination therapy with HP cream and adapalene 0.1% gel in comparison with the combination of BP 4% cream and adapalene 0.1% gel in the treatment of mild to moderate AV. METHODS: In a randomized, investigator-blinded trial, 52 patients (mean +/- SD age 25 +/- 6 years; 19 men and 33 women) with AV were randomly assigned to HP cream and adapalene gel (group HP + A) or to BP cream and adapalene gel (group BP + A), for eight consecutive weeks. Efficacy was assessed by total (TL), inflammatory (IL) and noninflammatory (NL) lesion counts performed at baseline and weeks 4 and 8. Tolerability was assessed by evaluating skin erythema, burning and dryness at weeks 4 and 8. RESULTS: All patients completed the study. At baseline, the mean +/- SD numbers of TL, IL and NL were 44 +/- 9, 25 +/- 7 and 19 +/- 6 in group HP + A and 40 +/- 9, 21 +/- 7 and 19 +/- 9 in group BP + A, respectively. At the end of the treatment period, TL, IL and NL were reduced by 93%, 92% and 95%, respectively, in group HP + A and by 88%, 86% and 90%, respectively, in group BP + A. A significantly (P = 0.0025) greater reduction in NL was observed in group HP + A in comparison with group BP + A. Tolerability was significantly better in group HP + A in comparison with group BP + A (P = 0.02). Skin dryness and burning sensation were more frequent in group BP + A. CONCLUSIONS: The combination of adapalene and HP cream is an effective topical treatment regimen in mild to moderate AV. This combination has shown a better tolerability profile in comparison with the combination of BP and adapalene.

Acne Vulgaris↗

Prevalence of symptoms experienced by patients with different clinical types of psoriasis.

BACKGROUND: The main dermatology textbooks describe only in passing pruritus in psoriasis and rarely mention other symptoms. A quantification of the presence of symptoms is not available for clinical subgroups of psoriasis. OBJECTIVES: To investigate the prevalence of symptoms experienced by patients with different clinical types of psoriasis. METHODS: The study was carried out in patients hospitalized for psoriasis between February 2000 and February 2002 at the inpatient wards of the Istituto Dermopatico dell'Immacolata, Rome, Italy. Symptoms were evaluated using the symptoms scale of Skindex-29. Clinical severity was assessed by the dermatologists using the Psoriasis Area and Severity Index (PASI), and by the patients completing the self-administered PASI. Psychiatric morbidity was evaluated using the 12-item General Health Questionnaire. RESULTS: In total, 936 eligible patients were analysed. The proportions of patients experiencing symptoms often or always in the 4 weeks before hospitalization were: 63.8% itching, 59.7% irritation, 46.1% burning/stinging, 39% sensitivity, 26% pain (from 10% in guttate psoriasis to 50% in arthropathic), 25.4% bleeding (17% pustular, 19% localized plaque, 36% palmoplantar), and 23.9% bothered by water (from 8.5% in the guttate form to 68% in palmoplantar). The prevalence of all symptoms was significantly higher in women and tended to increase with clinical severity. CONCLUSIONS: Our study provides evidence of the high frequency of a number of symptoms in different subgroups of psoriasis patients determined by their sociodemographic characteristics, clinical type and disease severity. Symptoms represent a serious disabling factor in patients affected by psoriasis, including those with low levels of psychological distress. Dermatologists should include symptoms in the evaluation of disease severity both in clinical practice and in clinical trials.

Adult↗

Squamous cell carcinoma arising in a venous ulcer as a complication of the Klippel-Trenaunay syndrome.

Klippel-Trenaunay (KT) syndrome is a vascular malformation characterized by a port-wine stain, varicose veins and hypertrophy of the affected limb. Ulceration is considered an uncommon complication of KT syndrome and occurrence of skin cancer has been previously reported only in one case. We observed a case of KT syndrome in a 48-year-old woman who developed a large ulcer and a squamous cell carcinoma on the affected leg.

Carcinoma, Squamous Cell↗

Genotypic and phenotypic characterization of Staphylococcus aureus strains isolated in subjects with atopic dermatitis. Higher prevalence of exfoliative B toxin production in lesional strains and correlation between the markers of disease intensity and colonization density.

Staphylococcus aureus strains generally colonize eczematous lesions of subjects with atopic dermatitis much more frequently than in the skin of normal individuals. The aim of this study was to provide a detailed genotypic and phenotypic analysis of S. aureus strains colonizing four different sites (lesional and non-lesional skin areas, nasal and pharyngeal mucosas) of 49 patients with atopic dermatitis. The 88 isolates were analyzed in duplicate by pulsed field gel electrophoresis and in their exfoliative toxin A or B production by latex test. The patients were characterized by age, sex, severity scoring of atopic dermatitis and serum eosinophil cationic protein. Fourteen (28.6%) of the patients were completely negative for S. aureus while 35 (71.4%) were positive in at least one site. The severity scores and eosinophil cationic protein levels were significantly correlated variables (P<0.001), linked to the colonization intensity (P ranging between 0.05 and <0.001 depending on the site) and to the number of colonized sites (P at least <0.01). The genotypic patterns, widely heterogeneous, showed no restriction to peculiar patterns. Only eight strains produced exfoliative toxin B which was significantly restricted to the lesional isolates (P=0.012).

Adolescent↗

Cutaneous carcinomas and preinvasive neoplastic lesions. Role of MMP-2 and MMP-9 metalloproteinases in neoplastic invasion and their relationship with proliferative activity and p53 expression.

BACKGROUND: Metalloproteinases (MMPs) are thought to be involved in the process of destruction of basement membranes and stromal invasion by neoplastic epithelial cells. AIMS: In order to investigate the role of MMPs in cutaneous oncogenesis we studied the expression of MMP-2 and MMP-9 in 34 cases of epidermal preinvasive neoplastic lesions and invasive carcinomas. We also studied their relationship with the expression of tissue inhibitors of MMPs and with proliferative activity and p53 expression in neoplastic epithelial cells. RESULTS: MMP-9 was found to be focally expressed by neoplastic epithelial cells at the infiltrative edges in microinvasive carcinomas and in dyskeratotic foci in Bowen's disease and widely invasive carcinomas. Gradation of Mib-1 positivity and p53 expression was found with increasing abnormality in the spectrum of malignancy. CONCLUSIONS: Our results seem to suggest the involvement of MMPs in microinvasive carcinomas, which show also low proliferative activity and p53 expression, whether other factors seem to be more important in widely invasive carcinomas.

Aged↗

Impaired ultraviolet-B-induced cytokine induction in xeroderma pigmentosum fibroblasts.

Xeroderma pigmentosum is a rare, autosomal recessive disease in which patients develop excessive solar damage at an early age and have a 1000-fold increased risk of developing cutaneous neoplasms. Xeroderma pigmentosum can be classified into seven complementation groups (A-G) with defects in different DNA nucleotide excision repair genes. Xeroderma pigmentosum patients also have impaired immune function including reduced natural killer cell activity and impaired induction of interferon-gamma. We hypothesized that altered cytokine induction may contribute to the immune defect in xeroderma pigmentosum patients. We examined cytokine mRNA expression after ultraviolet B irradiation using reverse transcriptase polymerase chain reaction in fibroblasts derived from five xeroderma pigmentosum patients in complementation groups A, C, and D and in complemented XP-A and XP-D cells. Cytokines interleukin-1beta and interleukin-6 displayed impaired ultraviolet B induction whereas interleukin-8 had normal induction in the xeroderma pigmentosum fibroblasts. Stable complementation of XP-A and XP-D cell lines increased ultraviolet-B-induced interleukin-1beta and interleukin-6 expression. These results demonstrate a deficient response of xeroderma pigmentosum fibroblasts to ultraviolet B in terms of cytokine interleukin-1beta and interleukin-6 induction but normal interleukin-8 induction and exhibit a role for DNA repair in cytokine induction.

Cell Line↗

Rethinking the role of tumour necrosis factor-alpha in ultraviolet (UV) B-induced immunosuppression: altered immune response in UV-irradiated TNFR1R2 gene-targeted mutant mice.

BACKGROUND: Ultraviolet (UV) B-induced immunosuppression, implicated in the pathogenesis of skin cancers, is postulated to be mediated in part by cis-urocanic acid (cis-UCA) via tumour necrosis factor (TNF)-alpha. TNF-alpha produces morphological changes in Langerhans cells indistinguishable from those induced by UVB exposure and antibodies against TNF-alpha have been demonstrated to inhibit UVB-induced immunosuppression in vivo. OBJECTIVES: To clarify further the role of TNF-alpha in UVB-induced immunosuppression and in cis-UCA immunosuppression. METHODS: We performed a contact hypersensitivity (CHS) assay on gene-targeted mutant mice (TNFR1R2-/-) lacking genes for both receptors (p55 and p75) for TNF-alpha. Mice were either irradiated with UVB or injected intradermally with cis-UCA, sensitized with 2,4-dinitrofluorobenzene, challenged on the ears and the response was measured. RESULTS: The TNFR1R2-/- mice showed hyporesponsiveness in the CHS response compared with wild-type (P < 0.001), confirming the proinflammatory role of TNF-alpha. However, significant suppression of CHS was seen after irradiation and after cis-UCA injection in both locally (sensitization on irradiated site; P < 0.05) and systemically (sensitization on non-irradiated site; P < 0.05) sensitized wild-type and gene-targeted mice. CONCLUSIONS: These results demonstrate that TNF-alpha signalling is only partially involved in UVB-induced immunosuppression and does not play a major part in the cis-UCA immunosuppression mechanism.

Animals↗

Role of Th2 cytokines, RANTES and eotaxin in AIDS-associated eosinophilic folliculitis.

The pathogenesis of AIDS-associated eosinophilic folliculitis is still unknown. The expression of chemokines and Th2-type cytokines is increased in other conditions associated with tissue eosinophilia and in allergic reactions. We evaluated the mRNA expression by reverse transcriptase polymerase chain reaction of two Th2 cytokines (interleukin-4 and interleukin-5) and of two chemokines (RANTES and eotaxin) in the skin of 6 patients with AIDS-associated eosinophilic folliculitis; the tissue localization of eotaxin was shown by immunohistochemistry. We demonstrated the increased expression of interleukin-4, interleukin-5, RANTES and eotaxin in lesional skin of the patients compared to normal skin of HIV+ individuals. We concluded that a Th2 pattern is present in AIDS-associated eosinophilic folliculitis. The cytokine milieu in this disease may favour a Th2 immune response to an unknown antigen, whereby RANTES and eotaxin act in synergy with interleukin-4 and interleukin-5 to mediate tissue inflammation.

Acquired Immunodeficiency Syndrome↗

Non-Herlitz junctional epidermolysis bullosa without hair involvement associated with BP180 deficiency.

Junctional epidermolysis bullosa (JEB) is a clinically and genetically heterogeneous recessively inherited blistering disease of the skin and mucous membranes due to impaired epithelial adhesion. In particular, defective expression of the 180-kD bullous pemphigoid antigen (BP180) has been correlated to a non-lethal (non-Herlitz) form of JEB, generalized atrophic benign epidermolysis bullosa (GABEB), characterized by widespread skin blistering healing with atrophy and by atrophic alopecia with onset in childhood. We report the case of a 33-year-old man suffering from a generalized blistering skin disorder since birth. He also presented nail dystrophy and tooth abnormalities. Mucosal involvement was limited to gingival erosion. Alopecia was absent and body, axillary and pubic hair were normal. Immunofluorescence analysis showed a markedly reduced expression of BP180, electron microscopy studies evidenced hypoplastic hemidesmosomes and Northern blot analysis confirmed a striking decrease in the amount of BP180 mRNA. The clinical features of our patient confirm that BP180 deficiency usually results in a non-Herlitz JEB form. However, the degree of skin, mucous membranes and hair involvement appears more variable and less typical than originally described for GABEB.

Adult↗

Immune modulation in pemphigus vulgaris: role of CD28 and IL-10.

Pemphigus vulgaris (PV) is an autoimmune bullous skin disease characterized by Abs to the desmosomal cadherin desmoglein-3. Although the autoantibodies have been shown to be pathogenic, the role of the cellular immune system in the pathology of pemphigus-induced acantholysis is unclear. To further delineate the potential role of T cell-signaling pathways in the pathogenesis of PV, we performed passive transfer experiments with PV IgG in gene-targeted mutant mice. Our results demonstrated that CD28-deficient mice (lacking a costimulatory signal for T cell activation) are 5-fold more sensitive to the development of PV than wild-type mice. To evaluate whether the higher incidence of disease was due to an impairment in intercellular adhesion of keratinocytes, we performed an in vitro acantholysis, using CD28-/- mice keratinocytes. No alteration in in vitro adhesion was detected in CD28-/--type keratinocytes. Because the CD28 molecule plays a pivotal role in the induction of Th2 cytokines, we examined the levels of a prototypic Th2 cytokine (IL-10) in CD28-/- mice. Lower levels of IL-10 mRNA were found in lesions from CD28-/- mice. To determine whether pemphigus susceptibility in CD28-/- was related to IL-10 deficiency, we performed passive transfer experiments in IL-10-/- mice that demonstrated increased blisters compared with controls. To confirm that IL-10 is involved in the pathogenesis, rIL-10 was given with PV IgG. IL-10 significantly suppressed the disease activity. These data suggest a potential role of IL-10 in PV.

Acantholysis↗

Expression of eotaxin, interleukin 13 and tumour necrosis factor-alpha in dermatitis herpetiformis.

BACKGROUND: The dermal and perivascular infiltrate in dermatitis herpetiformis (DH), which is mainly composed of CD4+ lymphocytes, neutrophils and eosinophils, is believed to play an important part in the pathogenesis of the disease. Previous studies suggest that cytokines such as interleukin (IL) -8, granulocyte-macrophage colony-stimulating factor, IL-4 and IL-5 could be involved in the pathogenesis of DH. These cytokines appear to drive tissue infiltration and maturation of eosinophils. Part of the effect of T-helper (Th) 2-type cytokines (IL-4, IL-5) on eosinophils could be mediated by eotaxin, which is a highly specific chemotactic protein induced by various cytokines [IL-4, IL-13, tumour necrosis factor (TNF) -alpha and interferon-gamma]. OBJECTIVES: To evaluate the expression of eotaxin and its inducers, IL-13 and TNF-alpha, in DH. METHODS We examined lesions collected from 10 DH patients with active disease. Sections from each specimen were incubated with anti-IL-13, anti-TNF-alpha and anti-eotaxin antibodies. Chloroacetyl esterase reaction was performed to show mast cell infiltration. RESULTS: Eotaxin was mainly expressed at the tips of the dermal papillae, within the microabscesses. Positivity was also found in the lymphomonocytic infiltrate in the dermis. IL-13 was expressed in the dermal infiltrate and TNF-alpha was found in the inflammatory infiltrate and in dermal vascular cells. CONCLUSIONS: These findings confirm the importance of the lymphomonocytic infiltrate and of Th2 cytokines in the pathogenesis of this disease, suggesting that tissue infiltration in DH is mediated by cell-specific chemokines such as eotaxin and not only by non-specific chemokines such as IL-8.

Adult↗

Huriez syndrome: case report with a detailed analysis of skin dendritic cells.

We report a 60-year-old man with familial scleroatrophic syndrome of Huriez who developed squamous cell carcinomas on the affected skin of the right palm. Immunohistochemical analysis showed a marked reduction in the number of CD1a+, Lag+ and S100+ epidermal Langerhans cells, but not of CD1b+ and factor XIIIa+ dermal dendritic cells, limited to palmoplantar skin. The Langerhans cell depletion was not associated with an abnormal skin content of mRNA for factors involved in Langerhans cell development or recruitment in the epidermis, including granulocyte/macrophage colony-stimulating factor, transforming growth factor-beta1 and macrophage inflammatory protein-3alpha. The results indicate that other as yet unknown mechanisms may account for the reduced number of Langerhans cells in the affected skin of such patients.

Carcinoma, Squamous Cell↗