Hypoalbuminemia, oliguria and peripheral cyanosis in an infant with severe atopic dermatitis

A six-month old male infant with severe atopic dermatitis was admitted with hypoalbuminemia, oliguria and cyanosis of the extremitie. s. There was marked edema and generalized eczema with foul, yellowish exudates. The patient's major clinical manifestations were attributed to the loss of albumin through the skin. Although atopic dermatitis is a common disease in children, here we want to show that systemic disturbances may arise from such condition, describe the total care given the patient, and emphasize the wholistic approach in managing cases of severe atopic dermatitis, intensive treatment was instituted and the patient was discharged after three weeks and remained in a stable condition.     

Prevalence of fatty liver in non-obese Japanese children with atopic dermatitis

Fatty liver in non-obese Japanese children was observed in 3.2% of non-atopic children and in 17.6% of patients with atopic dermatitis in 2000. The prevalence of fatty liver in non-obese children aged 0-12 years was studied from 2001 to 2003. Subjects were either non-atopic children, or suffering from bronchial asthma, allergic rhinitis, or atopic dermatitis. Fatty liver was studied by abdominal ultrasound scans. The prevalence of fatty liver was increasing annually, and it reached to 12.5% in non-atopic children, 13.1% in patients with bronchial asthma, 13.7% in patients with allergic rhinitis, or 33.9% in patients with atopic dermatitis, in 2003. Since fatty liver in childhood may be a risk factor for lifestyle-related diseases in future, care should be taken to prevent it.     

Breast milk fatty acid composition is associated with development of atopic dermatitis in the infant

OBJECTIVE: Breast milk fatty acids may have immunomodulatory properties related to the development of atopic disease. The aim of this study was to assess the impact of the breast milk fatty acid composition on the development of atopic dermatitis (AD) in high-risk infants. METHODS: Mothers with atopic disease were recruited at the end of gestation. Maternal food records and breast milk samples were collected at the infants' age of one month. Infants were clinically examined and AD diagnosed at one, three, six, and 12 months. RESULTS: Altogether 13 of 34 (38%) infants were diagnosed with AD during the first year of life. Infants developing AD had consumed breast milk with a higher ratio of saturated to polyunsaturated fatty acids and less n-3 fatty acids compared to infants not developing AD. Specifically, breast milk consumed by infants with AD contained more stearic acid, 8.9% of total fatty acids (95% confidence interval 7.9-10.0) in comparison to those without AD, 7.1% (95% CI 6.6-7.7). CONCLUSION: Breast milk rich in saturated and low in n-3 fatty acids may be a risk factor for atopic dermatitis in the infant.     

Expansion of activated eosinophils in infants with severe atopic dermatitis

Abstract   Background : There is increasing concern in Japan over infants with atopic dermatitis (AD) who present with severe systemic complications, such as hypoproteinemia, electrolyte disturbances, and delayed growth and development. They are often associated with extremely increased numbers of circulating eosinophils. However, the clinical significance of eosinophil expansion has not been thoroughly investigated.
Methods : This study attempted to determine the significance of eosinophilia and eosinophil activation in infant cases of AD by comparing multiple clinical parameters, indexes of eosinophil activation, and levels of serum cytokines. CD69 expression was determined by flow cytometry. The clinical severity of AD was graded by the severity SCORing of atopic dermatitis (SCORAD) method. Patients were classified into two groups, with and without CD69 on eosinophils. Nuclear lobes were evaluated under a microscopy. Serum levels of eosinophil-derived neurotoxin (EDN), interleukin (IL)-12, IL-18, IL-4, IL-5 and interferon (IFN)-γ were determined by enzyme-linked immunosorbent assay.
Results : Patients with CD69-positive eosinophils had significantly higher numbers of eosinophils and platelets, total IgE, and eosinophil nuclear lobes. They also showed growth failure, developmental delay, low serum albumin, and electrolyte disturbances. EDN and IL-18 levels were significantly increased in this group, but the levels of IL-4, IL-5 and IL-12 were not significantly different between the two groups. IFN-γ was not detectable in all patients with AD. Surface expression of CD69 indicates intense systemic allergic inflammation induced in severe cases of AD.
Conclusions : Evaluation of eosinophil activation and early therapeutic intervention is mandatory for the treatment of severe AD during infancy.     

Netherton syndrome: report of identical twins presenting with severe atopic dermatitis

We report the cases of 4-year-old identical twin sisters who presented with severe atopic dermatitis with intractable skin manifestations and multiple food allergies. Netherton syndrome (NS) (OMIM 256500) was suspected due to very high serum IgE levels, growth retardation, severe food allergies and typical hair finding (trichorrhexis invaginata). A definite diagnosis was made by genetic analysis. Our cases are unique in being the first identical twins with NS diagnosed by a novel mutation in the SPINK5 gene. NS should be considered in differential diagnosis in children who have generalized erythema with intractable eczematous lesions and elevated levels of IgE.     

Transient liver injury and severe neonatal cholestasis in infant with glucose-6-phosphate dehydrogenase deficiency due to a new mutation

We report the case of a neonate with a new, previously undescribed, glucose-6-phosphate dehydrogenase (G6PD) gene mutation, which was revealed by severe cholestasis, hyperbilirubinemia, and transient liver dysfunction. The severity of the clinical phenotype with ongoing chronic hemolytic anemia suggests that this mutation belongs to class 1 G6PD deficiency. The hemizygous mutation «c.675G>c; p.Trp225Cys» was detected by genomic sequencing. Since severe G6PD deficiency can be revealed by cholestasis, it is important to check G6PD enzyme activity when faced with a case of liver dysfunction in the neonatal period.     

Insulin-dependent diabetes mellitus and severe atopic dermatitis in a child with adenosine deaminase deficiency

We report a 2.3-year-old girl with complete lack of adenosine deaminase (ADA) activity who presented with severe atopic dermatitis and insulin-dependent diabetes mellitus but only mild recurrent infections. Abnormalities of immune function included profound depletion of CD8+ lymphocytes, hyperimmunoglobulinaemia E, and very low in vitro proliferative response to mitogens. Treatment with polyethylene glycol-conjugated ADA was followed by rapid amelioration of clinical and immunological conditions. The immunological and clinical features of this child suggest that the clinical spectrum of ADA deficiency may be broader than originally supposed.     

An infant with severe leucocyte adhesion deficiency

A case of severe leucocyte adhesion deficiency occurred in a 6 1/2-month-old boy whose parents were first-degree cousins. Evidence of the disease first became apparent with the late separation of the umbilical cord on the 20th day and with the later development of omphalitis. The most specific finding was the very low levels of CD18 and CD11, 0.44 and 0.15%, respectively. The boy died from sepsis which occurred as an extension of necrotic lesions on the ear and in the gluteal area.     

Methylprednisolone bolus: a novel therapy for severe atopic dermatitis

Seven children suffering from severe atopic dermatitis, unresponsive to standard therapy, received an iv bolus dose of methylprednisolone (20 mg/kg/day) for three days. Immunological parameters were evaluated before and after treatment. At the end of bolus therapy both skin lesions and itching improved for several months in five of seven patients. No side effects were observed, but a significant and transient lymphopenic response occurred, with lower CD4 + than CD8 + lymphocyte counts. Our data suggest that this therapy may be a novel and safe therapeutic approach in severe atopic dermatitis.     

Hypoproteinemia in severe childhood atopic dermatitis: A serious complication

As a complication of atopic dermatitis (AD), the incidence of hypoproteinemia is increasing among infants with severe AD in Japan. It can be a life‐threatening condition owing to hypovolemic shock as a result of hypoproteinemia and vascular infarction as a result of thrombocythemia. However, the pathophysiology of this condition remains unclear. The objectives of the present study were two‐fold. The first objective was to determine the main route of protein loss, i.e. through the damaged skin or the gastrointestinal tract, or as a result of insufficient food intake. The second objective was to identify whether allergy or infection was the cause of severe skin inflammation. Fifteen patients with AD were enrolled who had serum protein levels of 3.2–5.8 g/dl. Specific immunoglobulin E (IgE) and skin test to allergens, stool eosinophils, α₁‐antitrypsin clearance, skin Staphylococcus aureus colonization and superantigens (SAgs) produced by the organism, serum SAg‐specific IgE antibodies, serum interleukin (IL)‐5, IL‐6, IL‐12, and interferon‐γ (IFN‐γ) were evaluated. Prominent serous skin discharge was seen in all of the patients and was found to have almost the same protein concentration as serum. Marked thrombocytosis, with a maximum of 1,060 × 10³/ml, was seen. Skin culture revealed S. aureus colonization in all patients. SAg‐producing S. aureus were found in 84.6% of the patients. The concentration of serum IL‐5 was significantly increased and correlated well with the blood eosinophil count. Hence, the main route of protein loss was believed to be through damaged skin. The cause of severe inflammation was thought to be a combination of allergic inflammation and skin colonization by SAg‐producing S. aureus. Serum cytokines showed a T helper 2 (Th2) T‐cell‐mediated pattern. To prevent hypovolemic shock, vascular occlusion, and growth retardation, it is of vital importance to diagnose hypoproteinemia at an early stage and start appropriate therapy.     

Behavioral characteristics in 3- to 12-month-old infant with atopic dermatitis: a case–control study

There have been reports that refer to the personality of the patients with atopic dermatitis (AD) especially adult patients but there are few studies regarding the behavioral characteristics in AD infants. The aim of this study was to compare behavioral characteristics of 30 AD infants (3-12 months old) with 40 controls. The infants with the definite diagnosis of AD (according to Hanifin and Rajka criteria) referring to children medical center were included in this study. For assessing behavioral characteristics we used revised version of Infant Behavior Questionnaire for measuring 11 scales of behavioral characteristics (Fear, Perceptual Sensitivity, Distress to Limitations, Sadness, High Pleasure, Low pleasure, Approach, Rate of Recovery from Distress, Soothability, Smiling and Laugher, and Duration of Orienting). Questionnaires were filled out by the physicians with the cooperation of the parents. The AD group showed significantly higher scores in Perceptual Sensitivity, Soothability, and High Pleasure compared with control group (p = 0.000). In other characteristics no significant difference were noticed between atopic and non-patients. For eight characteristics, scores of atopic infants were similar healthy infants, but they tend to show more pleasure when subjected to an intense, novel or incongruity stimuli compared with healthy infants. Theoretically, higher scores in Perceptual Sensitivity, Soothability, and High Pleasure are concordant with the pervious studies about adrenomedullary system over activity.