Extracutaneous Manifestations of Neonatal Lupus Erythematosus

Cardiac NLE is the most frequently reported manifestation of NLE. Cardiac NLE has significant morbidity and mortality: approximately two thirds of the children with cardiac NLE require permanent pacemaker implantation, and there is 15%-20% mortality (Buyon et al. 1998, Eronen et al. 2000). The most commonly occurring lesion is third-degree heart block. Heart block almost always begins in utero during the second or third trimester, often presenting as a lesser degree of block that relatively quickly advances to complete heart block. Complete heart block is almost always permanent. Autopsy studies have shown replacement of the atrioventricular nodal area by fibrosis and calcification (Lee et al. 1987, Lev et al. 1971). Assuming that the pathologic findings are similar in surviving children, it is easy to understand why heart block persists despite resolution of disease activity.

In some children with complete heart block, cardiac muscle is involved as well. This is often evident shortly after birth, when correction of the low heart rate with pacemaker implantation fails to correct heart failure. However, heart failure has developed later during infancy in a few individuals, demonstrating the importance of close monitoring in children with cardiac NLE (Taylor-Albert et al. 1997).

Hepatobiliary disease of NLE apparently may assume several phenotypes. In a review of data from a national research registry, approximately 10% (19/219) of the cases in the registry had evidence of hepatobiliary disease (Lee et al. 2002). The three types of presentations noted were as follows: (a) liver failure in utero or shortly after birth, often having the phenotype of neonatal iron storage disease (also known as "neonatal hemochromatosis"); (b) transient conjugated hyperbilirubinemia occurring in the first few weeks of life; and (c) transient aminotransferase elevations, occurring at 2-3 months of age. The latter two presentations eventuate in complete resolution, with no apparent residua. It has not been shown conclusively that each of these presentations truly represents a manifestation of NLE, but based on currently available information it seems likely.

The cytopenia most commonly associated with NLE has been thrombocytopenia (Watson et al. 1988). Remarkably, 5 of 57 children with cutaneous NLE in the national registry had neutropenia (Neiman et al. 2000). The cytopenias are transient and usually not associated with morbidity. One child had a nonfatal episode of gastrointestinal bleeding attributed to thrombocytopenia (Lee et al. 1993).

How To Deal With Rosacea and Eczema

How To Deal With Rosacea and Eczema

Rosacea and Eczema are two skin conditions that are fairly commonly found throughout the world. Each of them is characterized by different features, and can be both discomfiting as well as result in undesirable appearance features. In a nutshell, theyre problems that many would want to deal with.

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