[Paraneoplastic subacute cutaneous lupus erythematosus]. / Paraneoplastischer subakut kutaner Lupus erythematodes.

Subacute cutaneous lupus erythematosus (SCLE) is a subtype of cutaneous lupus erythematosus characterized by high photosensitivity, the occurrence of annular or papulosquamous skin lesions located in body regions exposed to UV light, the presence of anti-Ro/SS­A antibodies, and mild systemic involvement, such as arthralgia and myalgia. Similar to other subtypes of cutaneous lupus erythematosus, certain trigger factors exist for the development of SCLE, such as exposure to UV light, cigarette smoking and drugs. Rheumatic diseases, such as dermatomyositis, have been known as paraneoplastic syndromes for a long time. In recent years, there has been an accumulation of publications on the association of SCLE with malignant diseases. This article reports the case of a 78-year-old female patient who was diagnosed with the concomitant development of SCLE and gastric carcinoma. In all older patients with SCLE, patients with widespread skin affection outside the UV-exposed body areas or patients with B­symptoms, the presence of a paraneoplastic SCLE should be considered and appropriate diagnostic steps should be initiated to screen for an associated neoplastic disease.

[Reticular erythematous mucinosis-A special subtype of cutaneous lupus erythematosus?] / Retikuläre erythematöse Muzinose ­ Sonderform eines kutanen Lupus erythematodes?

Reticular erythematous mucinosis (REM syndrome) is a rare skin disease that predominantly affects women. It is clinically characterized by flat, partly reticular, irregularly configurated pale erythema located in the region of the central chest and upper back. The skin alterations are usually asymptomatic or associated with slight pruritus or burning. Extracutaneous diseases and involvement of internal organs do not occur in REM syndrome. Histopathological features include perivascular and periadnexal lymphocytic inflammatory infiltrates as well as increased deposition of mucin in the dermis. Due to the similar histology to lupus erythematosus (LE) tumidus, it is discussed whether REM syndrome can be assigned to the spectrum of cutaneous LE or can be assessed as a lupus-like disease. Moreover, both conditions respond well to treatment with hydroxychloroquine.

[Diffuse hyperkeratotic papules of the lower abdomen and genital region in a 38-year old male patient]. / Diffuse hyperkeratotische Papeln am unteren Abdomen und im Genitalbereich bei einem 38­jährigen Patienten.

A patient with diffuse angiokeratomas of the lower abdomen and genital region was diagnosed with Fabry disease on the basis of genetic testing. Fabry disease is an X-linked lysosomal storage disease that can affect several organ systems including the heart or kidneys, resulting in reduced median survival. Pathogenetically, Fabry disease leads to a deficiency of the lysosomal enzyme α­galactosidase A (α-GAL A). Treatment options include lifelong enzyme replacement therapy or chaperone therapy.

Fas and Fas ligand expression in maternal blood and in umbilical cord blood in preeclampsia.

The Fas-Fas ligand (FasL) pathway of apoptosis is abnormally activated in diseases associated with impaired immune tolerance or chronic inflammation. Pregnancy-related hypertension is a spectrum of disease that commonly causes significant morbidity in women and in their newborn infants, is associated with generalized inflammation, and may be causally related to impaired maternal-fetal tolerance. Our recent observation of enhanced trophoblast expression of FasL in one form of pregnancy-related hypertension led us to hypothesize that this group of disorders might be associated with abnormal activation of the Fas-FasL pathway. To test this hypothesis, we prospectively quantified soluble and leukocyte-associated Fas receptor and FasL in the maternal and umbilical cord blood (CB) sera of 20 gestations complicated by preeclampsia and of 18 normal control gestations, using ELISA and flow cytometric analyses. We determined higher soluble FasL levels in paired maternal and CB sera of hypertensive gestations compared with control gestations (p < 0.01); in contrast, soluble Fas levels were similar between groups. Surface expression of FasL was lower on maternal (p < 0.01) and CB (p < 0.05) neutrophils from affected gestations, whereas surface Fas expression was lower on maternal (p < 0.02), but not CB, neutrophils and lymphocytes. We conclude that expression of Fas and FasL in sera and on leukocytes is altered in gestations complicated by preeclampsia, and speculate that activation of the Fas-FasL pathway mediates associated pathologic processes in affected women and in their neonates.

Data quality assurance, monitoring, and reporting.

In conclusion, the quality assurance and monitoring program is an integral and continuing part of study operations. A system must be devised and implemented by the coordinating center investigators, with the endorsement of the study leadership and support of the field site and resource center personnel. Proactive mechanisms for promoting high-quality data acquisition and reporting must be implemented. Data quality monitoring must address the entire process by which the data are gathered, transmitted, stored, and analyzed. Data quality should be monitored continually, with summary reports prepared and distributed to the study leadership. Appropriate training and certification enhance data quality, and site visits allow data collection and storage processes to be observed directly. The quality assurance and monitoring system must be documented. It should be flexible enough so that new means of quality assurance or monitoring can be added when necessary during the course of the study. At the completion of the study, quality monitoring results should be summarized in a final report regarding the level of quality achieved by the study investigators and personnel. Finally, for a quality assurance and monitoring program to be successful, the coordinating center investigators and personnel must provide prompt feedback and suggestions for corrective action whenever a data quality problem is discovered. This need can be met only when the coordinating center staff understand data quality goals and are up to date with all phases of data management and reporting. Delays in initiating any stage of data management and quality monitoring may result in uncorrectable data problems. Thus, knowledgeable and efficient coordinating center personnel are essential to achieving good data quality studywide.