Washing with water alone versus soap in maintaining remission of eczema.

BACKGROUND: There is no consensus on the use of soap in skin care for atopic dermatitis in Japan. Thus, this study aimed to evaluate the efficacy of soap to maintain eczema remission in atopic dermatitis patients during the fall-winter period in Japan. METHODS: This assessor-blinded, pragmatic randomized, non-inferiority study enrolled atopic dermatitis patients whose eczema was controlled by regular steroid ointment application less than or equal to 2 days / week (tacrolimus ointment was permitted). For 8 ± 3 weeks, participants washed their upper and lower limbs on one side with soap (soap side) and on the other side with water alone (water side). The primary outcome was an Eczema Area and Severity Index score at week 8 ± 3. RESULTS: Twenty-nine participants were analyzed. The Eczema Area and Severity Index scores at week 8 ± 3 of the water and soap sides were 0.0 (0.0-0.4) and 0.0 (0.0-0.4), respectively (P = 0.18). The difference between both sides was -0.02 (-0.11-0.08), and the limits of the 95% confidence interval did not reach the prespecified non-inferiority margin. The average Patient-Oriented Eczema Measure score was 1.27 ± 1.7 and 1.32 ± 1.8 for the water and soap sides, respectively (P = 0.92). The total number of additional steroid ointment applications was four (0-20) times and six (0-23) times, respectively (P = 0.98). Participants were categorized according to self-assessments of the usefulness of soap, with 2, 24, and 3 participants in the water-effective, invariant, and soap-effective groups, respectively. CONCLUSIONS: For children with controlled atopic dermatitis, washing with water alone was not inferior to washing with soap for maintaining remission of eczema during the fall-winter period in Japan.

Palmar hyperlinearity in early childhood atopic dermatitis is associated with filaggrin mutation and sensitization to egg.

BACKGROUND/OBJECTIVES: Palmar hyperlinearity is a typical clinical feature of Filaggrin gene (FLG) null mutations. There are reports of FLG mutations and allergic sensitization; however, reports on the relationship between palmar hyperlinearity to sensitization are limited. This study aimed to examine the association between palmar hyperlinearity and sensitization in atopic dermatitis (AD) children. METHODS: This cross-sectional, case-control study included children ˂ 6 years old with moderate-severe AD whose parents consented for mutation analysis and photographic documentation. Each child underwent genotyping to detect the eight most prevalent FLG mutations in the Japanese population: R501X, 3321delA, S1695X, Q1701X, S2554X, S2889X, S3296X, and K4022X. Clinical features and parameters including egg-specific IgE were examined, and palm photographs were evaluated by 12 trained dermatologists blinded to genotyping results. RESULTS: Of the 57 patients (age range, 2 months to 5 years; median, 22 months), 16 were heterozygotes and three were compound heterozygotes. Palmar hyperlinearity, as recognized by more than two-thirds of dermatologists, was significantly associated with FLG mutation (P = 0.002, OR = 6.98, 95% CI = 2.1-23.7), and this association was observed especially in children over 2 years. Cross-shaped crease of the thenar eminence, as known in previous reports, also demonstrated significant correlation with FLG mutation. When the children were divided according to the presence or absence of palmar hyperlinearity, the egg white-specific IgE was significantly higher in the hyperlinearity group (55.9 vs 18.3 IU/mL, P < 0.05). CONCLUSIONS: Palmar hyperlinearity indicates possible inherited barrier abnormalities of the skin in early childhood. Its identification may help to predict a more accurate prognosis, such as sensitization.

Immunodeficiency in Two Female Patients with Incontinentia Pigmenti with Heterozygous NEMO Mutation Diagnosed by LPS Unresponsiveness.

PURPOSE: Anhidrotic ectodermal dysplasia with immunodeficiency (EDA-ID) is caused by mutations in the NF-κB essential modulator (NEMO) or NF-κB inhibitor, alpha (IKBA) genes. A heterozygous NEMO mutation causes incontinentia pigmenti (IP) in females, while a hemizygous hypomorphic mutation of NEMO causes EDA-ID in males. In general, immunodeficiency is not shown in IP patients. Here, we investigated two female patients with IP and immunodeficiency. METHODS: The patients were initially suspected to have IRAK4 deficiency and Mendelian susceptibility to mycobacterial disease, respectively, because of recurrent pneumonia with delayed umbilical cord detachment or disseminated mycobacterial infectious disease. We measured tumor necrosis factor (TNF)-α production and performed mutation screening. RESULTS: The TNF-α production from lipopolysaccharide (LPS)-stimulated CD14-positive cells was partially defective in both female patients. A genetic analysis showed them to carry the heterozygous NEMO mutations c.1167_1168insC or c.1192C>T. Although NEMO mutations in IP patients are typically eliminated by X-inactivation skewing, an analysis of cDNA obtained from the somatic cells of the patients showed the persistence of these mutations in peripheral blood mononuclear cells and peripheral granulocytes. A NF-κB reporter gene analysis using NEMO-deficient HEK293 cells showed the loss of NF-κB activity in these NEMO mutants, while the NF-κB protein expression levels by the NEMO mutants were consistent with those of wild-type NEMO. CONCLUSIONS: The delayed skewing of the mutant allele may be responsible for the observed innate immune defect in these patients. The detection of LPS unresponsiveness is suitable for identifying female IP patients with immunodeficiency.

Usefulness of lymphocyte stimulation test for the diagnosis of intestinal cow's milk allergy in infants.

BACKGROUND: Bottle-fed infants sometimes develop intestinal cow's milk allergy (ICMA). Because cow's milk-specific IgE antibody (CM-IgE) levels are normal, the lymphocyte stimulation test (LST) has been proposed as an alternative diagnostic test for ICMA. The present study evaluated the diagnostic value of LST in a large number of patients with ICMA in Japan. METHODS: Ninety-six infants who developed intestinal symptoms after ingestion of cow's milk formula and showed remission of symptoms after elimination of this food were enrolled as patients with probable ICMA. Seventy-two subjects with normal CM-IgE levels and a positive result in an oral food challenge test (OFCT) for cow's milk formula were diagnosed with ICMA. Another 10 infants with normal CM-IgE levels and a negative OFCT result were diagnosed with nonspecific intestinal symptoms (NIS). The status of cell-mediated immunity against cow's milk proteins was estimated by LST for κ-casein. RESULTS: In the 72 patients with ICMA (38 boys and 34 girls), the median age at onset was 9 days. Sixty-two of 72 (86.1%) patients with ICMA tested positive in the LST for κ-casein. In contrast, only 2 of the 10 NIS infants tested positive. The incidence of a positive LST result was significantly higher in the ICMA group than in the NIS group (p < 0.0001). The area under the receiver-operating characteristic curve for this test was as high as 0.856. CONCLUSIONS: This study strongly suggests that the LST for κ-casein is a useful diagnostic test for ICMA.