Association between autoimmune thyroiditis and depressive disorder in psychiatric outpatients.

Thyroid diseases are often associated with psychiatric disorders. The prevalence of autoimmune thyroiditis in the general population is estimated to be at about 5-14 %. A clinical study was conducted to evaluate the association between autoimmune thyroiditis and depression in psychiatric outpatients.

Updated analysis of standardized photoprovocation in patients with cutaneous lupus erythematosus.

Objective: To determine the frequency and reproducibility of standardized photoprovocation in patients with cutaneous lupus erythematosus (CLE) and report our long-term experience.

Methods: Photoprovocation using a standardized protocol was evaluated retrospectively in 566 patients. A diagnosis of CLE was clinically and/or histologically confirmed in 431 patients, and 315 patients with polymorphic light eruption (PLE) were additionally included as controls.

Photoprovocation in cutaneous lupus erythematosus: a multicenter study evaluating a standardized protocol.

Photosensitivity is an important and distinguishing sign in various subtypes of cutaneous lupus erythematosus (CLE); however, it remains poorly defined. The purpose of this study was to evaluate whether standardized photoprovocation is a reproducible method to assess photosensitivity in subjects with CLE. A total of 47 subjects with CLE (subacute cutaneous lupus erythematosus (SCLE), n=14; discoid lupus erythematosus (DLE), n=20; lupus erythematosus tumidus (LET), n=13) and 13 healthy volunteers underwent photoprovocation at seven European sites.

Efficacy of tacrolimus 0.1% ointment in cutaneous lupus erythematosus: a multicenter, randomized, double-blind, vehicle-controlled trial.

Background: Topical calcineurin inhibitors are licensed for the treatment of atopic dermatitis; however, the efficacy of tacrolimus in cutaneous lupus erythematosus (CLE) has only been shown in single case reports.

Objective: In a multicenter, randomized, double-blind, vehicle-controlled trial, we sought to evaluate the efficacy of tacrolimus 0.1% ointment for skin lesions in CLE.

Nodular lesions of choroid plexus in Hurler disease.

Neuropathologic examination of six brains from children with Hurler disease revealed nodular lesions in the glomus of choroid plexus caused by proliferation of vacuolated pericytes, fibroblasts, and arachnoid cells on the background of collagenized and myxoid stroma. This localization of lesions can be explained by the presence of a rich vascular network, as well as cellular heterogeneity greater in the glomus than in other parts of the choroid plexus or in the brain parenchyma. The development of nodules did not correlate with the age, severity of hydrocephalus, or the degree of expansion of the perivascular spaces in the brain.

Photoprotective effects of a broad-spectrum sunscreen in ultraviolet-induced cutaneous lupus erythematosus: a randomized, vehicle-controlled, double-blind study.

Objective: We sought to assess if the exclusive use of a broad-spectrum sunscreen can prevent skin lesions in patients with different subtypes of cutaneous lupus erythematosus (CLE) induced by ultraviolet (UV) irradiation under standardized conditions.

Methods: A total of 25 patients with a medical history of photosensitive CLE were included in this monocentric, randomized, vehicle-controlled, double-blind, intraindividual study. The test product and its vehicle were applied 15 minutes before UVA and UVB irradiation of uninvolved skin areas on the upper aspect of the back in a random order, and standardized phototesting was performed daily for 3 consecutive days.

Reduction of regulatory T cells in skin lesions but not in peripheral blood of patients with systemic scleroderma.

Objective: To determine the frequency and suppressive capacity of regulatory T cells (T(reg)) and their association with clinical parameters in patients with systemic scleroderma (SSc).

Methods: Peripheral blood from 25 patients with SSc, 15 patients with localised scleroderma (LS) and 29 healthy controls (HC) was studied. Analysis of CD4(+) forkhead box P3 (Foxp3)(+) and CD4(+)CD25(++)Foxp3(+) T(reg) subpopulations was carried out by flow cytometry and cell proliferation was quantified by (3)H-thymidine incorporation.

Evaluation of disease activity and damage in different subtypes of cutaneous lupus erythematosus using the CLASI.

Background: The Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) is a scoring system for patients with cutaneous lupus erythematosus (CLE) to assess disease activity and damage. Objective  The aim of this study was to evaluate whether the CLASI is a useful instrument which reflects the different subtypes of CLE comparably well in each parameter.

Methods: A total of 50 patients (42 female, 8 male) with different subtypes of CLE, including acute CLE (ACLE), subacute CLE (SCLE), chronic CLE (CCLE) and LE tumidus (LET), from the Departments of Dermatology, University of Düsseldorf, Germany, and Danderyd Hospital, Stockholm, Sweden, were evaluated using the CLASI at one time point.

Anti-annexin 1 antibodies: a new diagnostic marker in the serum of patients with discoid lupus erythematosus.

Annexin 1 is an anti-inflammatory molecule and has also been described to be a common target of autoantibodies. In this study, we determined whether antibodies against annexin 1 can be detected in sera of patients with cutaneous lupus erythematosus (CLE). Levels of anti-annexin 1 antibodies were evaluated by a new established enzyme-linked immunosorbent assay and found to be significantly higher in sera of patients with CLE when compared to normal healthy donors (NHD).

A database analysis of cutaneous lupus erythematosus with the EUSCLE Core Set Questionnaire.

The aim of this study was to determine whether the Core Set Questionnaire developed recently by the European Society of Cutaneous Lupus Erythematosus (EUSCLE) is a useful tool to evaluate clinical features and therapeutic strategies in cutaneous lupus erythematosus. Disease characteristics were analysed in 50 patients with different subtypes of cutaneous lupus erythematosus from two European centres (Germany and Sweden). Mean age at onset of disease was 42.

Revised Cutaneous Lupus Erythematosus Disease Area and Severity Index (RCLASI): a modified outcome instrument for cutaneous lupus erythematosus.

Background: In 2005, a scoring system (CLASI, Cutaneous Lupus Erythematosus Disease Area and Severity Index) was developed for patients with cutaneous lupus erythematosus (CLE) to assess disease 'activity' and 'damage'. However, the CLASI does not give an accurate assessment of the severity in all disease subtypes.

Objectives: The main objective of this study was to analyse critically the included parameters of the CLASI and to revise the activity and damage score taking into account various clinical features of the different subtypes of CLE.

Effect of bosentan on skin fibrosis in patients with systemic sclerosis: a prospective, open-label, non-comparative trial.

Objectives: To assess the effect of the ET-receptor antagonist bosentan on skin fibrosis and functionality in patients with SSc.

Methods: In this prospective, open-label, non-comparative trial, a total of 10 patients with SSc received 62.5 mg of bosentan twice daily for 4 weeks and then 125 mg twice daily for 20 weeks.

Lupus erythematosus tumidus is a separate subtype of cutaneous lupus erythematosus.

Background Lupus erythematosus tumidus (LET) is a rare disease which was first described in 1909 but has not always been considered as a separate entity of cutaneous lupus erythematosus (CLE) in the international literature. Objectives To compare characteristic features of different subtypes of CLE and to analyse whether LET can be distinguished as a separate entity in the classification system of the disease. Methods The study involved 44 patients with CLE, including 24 patients with LET, 12 with discoid lupus erythematosus (DLE) and eight with subacute CLE (SCLE), from two centres in Germany.

Fixed solar urticaria with delayed onset.

Fixed solar urticaria (FSU) is an extremely rare type of solar urticaria characterized by urticarial wheals appearing frequently confined to fixed areas of the skin. After a few minutes of exposure to sunlight or other sources of radiation, urticarial lesions can usually be induced exclusively in the same localization. We report a case of delayed onset FSU occurring 6 hours after exposure to ultraviolet A and B light.

Development of a Core Set Questionnaire by the European Society of Cutaneous Lupus Erythematosus (EUSCLE).

A study group of the European Society of Cutaneous Lupus Erythematosus (EUSCLE) developed a Core Set Questionnaire for the evaluation of patients with cutaneous lupus erythematosus (CLE). The aim of the EUSCLE Core Set Questionnaire is to gain a broad and comparable data collection of patients with CLE from different European centers, to achieve consensus concerning evidence-based clinical standards for disease assessment, and to develop diagnostic and therapeutic guidelines. The authors designed the EUSCLE Core Set Questionnaire by including parameters considered most relevant for the evaluation of CLE and compiled from international literature, clinical praxis, and long-term experience with this disease.

The registry of the German Network for Systemic Scleroderma: frequency of disease subsets and patterns of organ involvement.

Objective: Systemic sclerosis (SSc) is a rare, heterogeneous disease, which affects different organs and therefore requires interdisciplinary diagnostic and therapeutic management. To improve the detection and follow-up of patients presenting with different disease manifestations, an interdisciplinary registry was founded with contributions from different subspecialties involved in the care of patients with SSc.

Methods: A questionnaire was developed to collect a core set of clinical data to determine the current disease status.

[Current diagnosis of cutaneous lupus erythematosus].

Cutaneous lupus erythematosus (CLE) is a disease with different subtypes and the new classification system includes acute CLE (ACLE), subacute CLE (SCLE), chronic CLE (CCLE), and intermittent CLE (ICLE). The broad spectrum of skin involvement and the possibility of systemic organ manifestations at the beginning and during the course of CLE require specific diagnostic procedures. Clinical assessment of the cutaneous manifestations is necessary along with a detailed patient's history.

Aspartylglucosaminuria in a Canadian family.

Aspartylglucosaminuria (McKusick 208400) is a lysosomopathy associated with aspartylglucosaminidase (L-aspartamido-beta-N-acetylglucosamine amidohydrolase, EC 3.5.1.

Hyperornithinemia-hyperammonemia-homocitrullinuria (HHH)-syndrome. Ultrastructural changes of mitochondria in cultured dermal fibroblasts of three patients.

Mitochondria of fibroblasts cultured from the skin obtained at biopsy from three patients with the hyperornithinemia-hyperammonemia-homocitrullinuria (HHH)-syndrome, one of the autosomal recessive, heritable urea cycle disorders, were studied with appropriate controls ultrastructurally. The patients were two severely retarded 10- and 12-year-old boys, and a 22-year-old sister of the former whose mental status was at the low normal range; she never had motor impairments or seizures. The mitochondria, similar in all three patients, were increased in number, very long, branching and/or "looping," and tortuous.