Neonatal lupus erythematosus is an uncommon syndrome, which is caused by transplacental passage of maternal autoantibodies to Sjögren's syndrome A or B autoantigens. The clinical presentation includes distinctive cutaneous lesions resembling those seen in systemic lupus erythematosus, hepatobiliary disease, and cytopenias, which disappear with the clearance of maternal autoantibodies. The most severe presentation is a total atrioventricular heart block, which begins during the second trimester of gestation and is irreversible. The risk of having a child with neonatal lupus erythematosus in mothers who test positive for autoantibodies to Sjögren's syndrome autoantigens is approximately 2% for first pregnancies or if previous babies were healthy. The risk increases by approximately tenfold if a previous child had neonatal lupus erythematosus syndrome. The diagnosis of neonatal lupus erythematosus is made when the mother has autoantibodies to Sjögren's syndrome autoantigens, and the fetus or newborn develops atrioventricular heart block, or the newborn develops the typical rash or hepatic or hematologic manifestations in the absence of other explanation. Fetal echocardiography from the 16th to the 26th week of gestation is advised in mothers with autoantibodies to Sjögren autoantigens. The detection of a slow fetal heart rate or the postnatal diagnosis of atrioventricular heart block warrants immediate maternal testing for these autoantibodies if not previously tested.

Transient benign hyperphosphatasemia is likely the most common cause of hyperphosphatasemia among healthy infants and toddlers. Sometimes it also occurs in older children and adults, indicating that the traditional term transient benign hyperphosphatasemia of infancy and early childhood may not be correct. The elevation in alkaline phosphatase persists for >4 months in ≈20% of the cases. Recognition of this benign condition is crucial to avoid unnecessary investigations.

In childhood, cutaneous small-vessel vasculitides include Henoch-Schönlein syndrome, a systemic vasculitis, and Finkelstein-Seidlmayer syndrome, a skin-limited vasculitis. Both Henoch-Schönlein and Finkelstein-Seidlmayer syndromes are seen more frequently in white or Asian compared with black children and occur especially in winter and spring with a male-to-female ratio of approximately 2:1. In everyday clinical practice, both conditions are diagnosed on clinical grounds without histological confirmation. The characteristic cutaneous hallmarks of Henoch-Schönlein syndrome include a purpuric rash in all and a subcutaneous edema in approximately every second case, which are often preceded by non-specific red or pink macular elements that mimic a non-itching urticarial rash. Recent data point out that Henoch-Schönlein children often present further cutaneous findings such as Köbnerization, Rumpel-Leede capillary fragility phenomenon, and blistering eruptions. Children with Finkelstein-Seidlmayer syndrome are usually ≤24 months of age and not ill-appearing. They present with (a) large, round, red to purpuric plaques (often with a targetoid appearance) predominantly over the cheeks, ears, and extremities and (b) often tender non-pitting edema of the distal extremities, ears, and face (without pruritus). Both in Henoch-Schönlein syndrome and Finkelstein-Seidlmayer syndrome, there is often scrotal involvement. The cutaneous findings remit without sequelae within 2 months in Henoch-Schönlein and 3 weeks in Finkelstein-Seidlmayer syndrome.

Aim: Fever phobia describes exaggerated concerns about the consequences of childhood fever and broader awareness is needed in everyday clinical practice. We investigated the factors associated with fever phobia in caregivers and healthcare providers and the geographical distribution of the issue. Methods:The National Library of Medicine, Excerpta Medica and Google Scholar databases were searched. Results:We retrieved 76 papers, published in English from 1985 to 2018, which covered wide areas of Asia, Europe, America, Africa and Australia. The occurrence of fever phobia was confirmed in 65 papers covering 26 521 caregivers. A number of factors were significantly associated with fever phobia, including low educational or socioeconomic levels, a history of febrile seizures in the child and young maternal age. Fever phobia was also more common in Bedouins and in people from Latin America, Southern Italy and Turkey. There were also 15 papers that addressed fever phobia among 4566 healthcare providers. All the reports suggested that a fear of fever and a tendency to over treat was common among physicians and nurses.Conclusion: Fever phobia was a common world phenomena that affected caregivers and healthcare providers. SUPPORTING INFORMATIONAdditional supporting information may be found online in the Supporting Information section at the end of the article:Appendix S1 References from S61 to S88.

No abstract

In previously healthy subjects, vulvar ulcers are mostly caused by sexually transmitted microorganisms. Lipschütz's acute vulvar ulceration, first reported in 1912, is a non-sexually acquired condition characterized by sudden onset of a few genital ulcers. We systematically review presentation, underlying causes and disease duration of Lipschütz's ulceration.Comprehensive source of Excerpta Medica, National Library of Medicine and Web of Science databases was performed. Reports including cases of apparently previously healthy females affected by Lipschütz's ulceration were selected a predefined database was used to extract data on demographics, history, clinical and microbiological findings, and treatment.The search disclosed 158 cases. Almost 90% of cases were ≤20 years of age and sexually inactive. Lesions were usually one to about three, painful, ≥10 mm large, well-delimited, with a fibrinous and necrotic center and a symmetric distribution. Voiding disorders and enlarged inguinal lymph nodes were observed in a large subset of cases. Canker sores were noted in 10% of patients. Lipschütz's vulvar ulceration occurred concomitantly with an infectious disease in 139 cases. Infectious mononucleosis syndrome (N=40) was the most frequently detected well-defined infection, followed by mycoplasma species infections (N=11). The disease resolved after ≤3 weeks. CONCLUSIONS: Lipschütz's ulceration mainly affects both sexually inactive and, less frequently, sexually active subjects ≤20 years of age, presents with ≤3 vulvar ulcers, resolves without recurrences within 3 weeks and is temporarily associated with an infection, most frequently a flu-like illness or an infectious mononucleosis syndrome.

No abstract

In humans with short bowel syndrome (or carbohydrate malabsorption), D-lactic acidosis is likely rather common and under-recognized. This condition should be included in the differential diagnosis of unexplained high-gap metabolic acidosis where the anion causing the acidosis is not known. Furthermore, diabetic acidosis might be caused by accumulation of both ketone bodies and D-lactic acid. Finally, there are endogenous sources of D-lactic acid in subjects with propylene glycol intoxication.