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We investigated [sup 99m] Tc-tetrofosmin scintigraphy in 27 patients with Kaposi’s sarcoma: 20 had classic (CK), 5 AIDS-associated (AK) and 2 transplantation-associated (TK) variants. Twenty-three patients had clinically evident cutaneous and/or mucosal lesions, 9 of them with associated sarcomatous lymphadenopathy; 2 TK patients had only lymph nodes or other extracutaneous Kaposi sites. Both planar and SPECT [sup 99m] Tc-tetrofosmin scintigraphies were performed in all cases and neck pinhole (P)-SPECT in selected patients. [sup 99m] Tc-tetrofosmin uptake was observed in 88% of patients with clinically evident cutaneous and/or extracutaneous Kaposi lesions. Scintigraphy gave additional information on cutaneous lesion extent, particularly SPECT regarding deep invasion and subclinical sites in some cases. However, scintigraphy was less sensitive in the detection of small, isolated and scattered lesions. SPECT/P-SPECT were positive in 8/8 patients with sarcomatous lymph nodes, planar imaging in 5/8, ultrasonography in 7/8, while all procedures were negative in 6 other patients with reactive or HIV infection lymph nodes. SPECT demonstrated lymphadenopathy remission in 1 TK patient after immunosuppressive therapy modification and, like planar imaging, ascertained an associated lymphoma with [sup 67] Ga-citrate combined. [sup 99m] Tc-tetrofosmin scintigraphy, especially SPECT, can be useful both in the detection and staging of Kaposi sarcoma lesions as a complementary tool to clinical and other conventional diagnostic methods. Copyright © 2003 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

HLA-A, B, Cw, DR and DQ antigens were serologically determined in 105 patients suffering from lichen planus (LP). Of these patients, 87 bad idiopatbic LP and 18 bad secondary LP. In the first group, 43 bad cutaneous LP without mucosal lesions, 17 bad cutaneous LP with mucosal lesions and 27 bad purely mucosal LP. No HLA antigen was found to be significantly associated with secondary LP or with mucosal idiopathic LP. In cutaneous idiopathic LP with or without mucosal lesions, the HLA-DR1 and DQI antigen frequency was significantly increased, and that of HLA-DQ3 significantly decreased. Among the HLA-DR1 cutaneous idiopathic LP patients, 78.5% carried the DRB1*0101 allele, and 21.4% the DRB1*0102 allele, compared with 35-7 and 67-8%, respectively, of the HLA-DR1 controls. Our data demonstrate that idiopathic LP is influenced by HLA-associated genetic susceptibility and resistance factors not involved in secondary LP, and that cutaneous idiopathic LP is a genetically and therefore pathogenetically different condition from purely mucosal idiopatbic LP. [ABSTRACT FROM AUTHOR]

Case reports Five cases of palm and sole lichen planus (LP) taken from our series of 263 cases observed from 1985 to date are reported. Comments The clinical features of LP on the palms and soles are hyperkeratotic papules, erythematous patches, erosive lesions and also acrosyringeal LP. It has been hypothesized that beta-blocker drugs excreted by the eccrine if glands could influence the physiology of the excretory duct producing the acrosyringeal modification of LP. The authors evidence the association, in two patients, between erosive palm and sole LP and HCV-positive hepatopathy and also the association between LP erosive features and liver involvement. [ABSTRACT FROM AUTHOR]

Focuses on the study of matrix metalloproteinases (MMS) in Kaposi's sarcoma (KS). Characteristics of Kaposi's sarcoma; Role of KS inhibition of MMS activity in reducing tumor growth and migration; Assessment of immunohistochemistry of samples from patients with KS.

Discusses the need to take more note of the possibility of discoid lupus erythematosis in children. Call for such cases to be subjected to close clinical and hematological follow-up; Evaluation of the likelihood of evolution into systemic lupus erythematosus.

Describes a case of discrete papular lichen myxedematosus (LM) in a patient affected by virus C hepatopathy. History of papular lesions on the clavicle, neck, face and scalp; Extremely itchy lesions with a burning sensation; Modest hyperkeratosis of the epidermis; Accumulation of mucinous material in the reticular and middle dermis; Difficulty in treating LM.

Antiphospholipid syndrome is an immune-mediated acquired disorder characterized by vein and/or artery thromboses and obstetrical complications associated with the presence of antiphospholipid antibodies in the blood. aPL include anticardiolipin antibodies (aCL), antiβ2glycoproteinI (antiβ2GPI), and lupus anticoagulant (LAC). A primitive idiopathic form and a secondary form of APS are recognized. The latter, observed mostly in patients affected by systemic lupus erythmatosus (SLE), has been described in numerous other autoimmune diseases. The association between CDLE and APS in not frequent. Indeed, although the literature contains several reports of CDLE patients positive for aPL, only two of these present sufficient clinical and laboratory characteristics for a diagnosis of APS..We present a case of APS and CDLE present contemporaneously in the patient. In our case, CDLE arose after the diagnosis of APS. Therefore, ours constitutes the first case of CDLE in an APS-diagnosed patient.

Background: Lipodystrophies are a large heterogeneous group of genetic or acquired disorders characterized by generalized or partial fat loss, usually associated with metabolic complications such as diabetes mellitus, hypertriglyceridemia and hepatic steatosis. Many efforts have been made in the last years in identifying the genetic etiologies of several lipodystrophy forms, although some remain to be elucidated., Methods: We report here the clinical description of a woman with a rare severe lipodystrophic and progeroid syndrome associated with hypertriglyceridemia and diabetes whose genetic bases have been clarified through whole-exome sequencing (WES) analysis., Results: This article reports the 5th MDPL (Mandibular hypoplasia, deafness, progeroid features, and lipodystrophy syndrome) patient with the same de novo p.S605del mutation in POLD1. We provided further genetic evidence that this is a disease-causing mutation along with a plausible molecular mechanism responsible for this recurring event. Moreover we overviewed the current classification of the inherited forms of lipodystrophy, along with their underlying molecular basis., Conclusions: Progress in the identification of lipodystrophy genes will help in better understanding the role of the pathways involved in the complex physiology of fat. This will lead to new targets towards develop innovative therapeutic strategies for treating the disorder and its metabolic complications, as well as more common forms of adipose tissue redistribution as observed in the metabolic syndrome and type 2 diabetes., (Copyright © 2015 Elsevier Inc. All rights reserved.)

It is widely accepted that a deranged immune system plays a key role in the onset and evolution of classic Kaposi sarcoma (CKS). Nevertheless, the usage of the T-cell receptor (TCR) β-variable (BV) chain repertoire expressed by peripheral blood lymphocytes in patients with CKS is still unknown. With the aim of providing some further insights into the complex role of the immune system in CKS pathogenesis, we performed an extensive analysis of the TCR BV repertoire in both CD4(+) and CD8(+) T cells in 30 human herpesvirus 8-positive Sardinian patients with CKS and an equal number of age-matched healthy controls. We used a panel of monoclonal antibodies covering approximately 70% of human BV subfamilies and third complementarity determining region (CDR3) spectratyping. Patients with CKS showed an increased frequency of BV expansions in both CD4(+) and CD8(+) lymphocytes, with no prevalent clones. On spectratyping analysis, most of the 720 BV CDR3 profiles obtained from both CD4(+) and CD8(+) T cells in patients with CKS were skewed. In particular, the surprising increase of BV skewing observed in CD4(+) lymphocytes mimics the pattern of progressive TCR BV narrowing described in responses to persistent viral antigen stimulations. Our findings support the hypothesis that CKS evolution is associated with inadequate activation rather than impairment of the immune system.