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To report 2 cases of Iso-Kikuchi syndrome, both of which were congenital. Case 1, a 7-month-old female infant, visited the hospital due to abnormalities in the nail plate of the left index finger for 7 months; case 2, a 3-year-old male child, also visited the hospital due to abnormalities in the nail plate of the left index finger for 3 years. The 2 patients both presented with 2 tiny, independent nail plates on both sides of the nail bed of the left index finger, instead of normal nail plates. Based on their clinical features, the 2 patients were diagnosed with Iso-Kikuchi syndrome (congenital onychodysplasia of the index finger). The mother of case 1 had a history of progesterone use to prevent miscarriage during pregnancy, the mothers of the two patients both suffered from hypothyroidism and continued to receive thyroid hormone replacement therapy during pregnancy, and the mother of case 2 had a history of gestational diabetes. It is still unclear whether these comorbidities and drugs are directly related to the occurrence of Iso-Kikuchi syndrome.
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Objective:To evaluate clinical efficacy and safety of topical compound oleum lithospermi in the treatment of mild to moderate diaper dermatitis.Methods:A multicenter, randomized, positive-drug parallel-controlled clinical trial was conducted in 19 hospitals from July 2019 to August 2020. Children aged 0 - 12 months with mild to moderate diaper dermatitis were enrolled and randomly divided into 2 groups using a random number table: test group topically treated with compound oleum lithospermi, and control group topically treated with zinc oxide cream. The treatment was carried out 6 - 8 times a day for 7 days. Visits were scheduled on days 0 and 7, and total response rate and clinical healing time were evaluated. Changes in the dermatitis family impact (DFI) score were compared between the test group and control group, and adverse events were recorded. Statistical analysis was carried out by using independent-sample t test for normally distributed continuous data, Wilcoxon rank sum test for non-normally distributed continuous data, and chi-square test or Fisher′s exact test for unordered categorical data; survival curves were drawn, and log-rank test was used for comparisons between two groups. Results:A total of 343 children with diaper dermatitis were enrolled in this study. Among them, 31 children violated the protocol, so 312 were included in the per protocol set, including 157 in the test group and 155 in the control group, and all completed the visits on days 0 and 7. The total response rate was significantly higher in the test group (87.26%, 137/157) than in the control group (78.71%, 122/155; χ2 = 4.04, P = 0.044) . The clinical healing time was significantly shorter in the test group (5.33 days) than in the control group (6.13 days; χ2 = 4.67, P = 0.025) . After 7-day treatment, the DFI score significantly decreased in both the 2 groups compared with that before the treatment, but there was no significant difference in the DFI score between the 2 groups (test group: 4.02 ± 6.96, control group: 3.58 ± 5.90, Z = -0.39, P = 0.686) . The incidence of adverse events was 2.92% (5/171) and 5.45% (9/165) in the test group and control group respectively, and there was no significant difference between the 2 groups ( χ2 = 0.03, P = 0.865) . Conclusion:Compound oleum lithospermi can markedly reduce the clinical severity of diaper dermatitis, improve the total response rate, shorten the clinical treatment period, and improve the quality of life of children′s families with a favorable safety profile.
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Objective@#To investigate whether topical mucopolysaccharide polysulfate (MPS) cream can reduce the incidence of eczema and skin atrophy in patients with moderate- or low-risk infantile hemangioma after the treatment with topical beta-blockers or 595-nm pulsed dye laser (PDL) , and to analyze factors influencing the occurrence of eczema and skin atrophy.@*Methods@#A total of 722 patients aged 0- 1 years with moderate- or low-risk infantile hemangioma were enrolled from 5 Children′s Hospitals in China. According to the disease condition and therapy acceptability, these patients were divided into 6 groups to be treated with topical beta-blockers and MPS cream (blocker+ MPS group) , topical beta-blockers (blocker group) , 595-nm PDL and topical MPS cream (PDL+ MPS group) , 595-nm PDL (PDL group) , 595-nm PDL combined with topical beta-blockers and MPS cream (PDL+ blocker+ MPS group) , and 595-nm PDL and topical beta-blockers (PDL+ blocker group) , respectively. All the externally applied agents were applied twice a day, and PDL was performed once every 4 weeks. Efficacy and adverse reactions were evaluated after 3-month treatment. Univariate and multivariate Logistic regression analyses were carried out to analyze factors influencing the incidence of eczema and skin atrophy in patients with infantile hemangioma after treatment, and chi-square test was carried out to compare efficacy among the groups.@*Results@#After 3-month treatment, multivariate Logistic regression analysis for comparing the blocker+ MPS group with blocker group showed that the risk factor for eczema on the surface of hemangiomas was no topical treatment with MPS cream (P= 0.007, OR= 3.887, 95% CI: 1.439-10.493) , while no correlations were observed between the occurrence of skin atrophy on the surface of hemangiomas and analyzed factors. Multivariate Logistic regression analysis for comparing the PDL+MPS group with PDL group showed that no topical treatment with MPS cream (P < 0.001, OR= 7.402, 95% CI: 2.604-21.042) and northern areas (P < 0.001, OR= 67.048, 95% CI: 7.977-563.518) were risk factors for eczema on the surface of hemangiomas, and risk factors for skin atrophy on the surface of hemangiomas included no topical treatment with MPS cream (P = 0.001, OR = 9.371, 95 CI: 2.590 - 33.900) and abundant blood supply of hemangiomas (P = 0.036, OR = 2.971, 95% CI: 1.075-8.208) . Multivariate Logistic regression analysis for comparing the PDL + blocker+ MPS group with PDL+ blocker group showed that risk factors for eczema on the surface of hemangiomas were no topical treatment with MPS cream (P= 0.005, OR= 3.426, 95% CI: 1.446-8.119) and northern areas (P < 0.001, OR= 31.704, 95% CI: 6.924-145.158) , and risk factors for skin atrophy on the surface of hemangiomas included no topical treatment with MPS cream (P < 0.001, OR= 6.011, 95% CI: 2.558-14.126) and southern areas (P= 0.022, OR= 3.021, 95% CI: 1.177-7.753) . After 3-month treatment, the response rate was significantly higher in the PDL group than in the PDL+ MPS group (χ2= 4.531, P= 0.033) , and significantly higher in the blocker group than in the blocker+ MPS group (χ2= 4.344, P= 0.037) . There were no significant differences in the response rate or cure rate among the other groups (all P > 0.05) .@*Conclusion@#During the treatment of moderate- or low-risk infantile hemangioma with topical beta-blockers or 595-nm PDL, the combination with topical MPS cream can reduce the occurrence of eczema and skin atrophy without affecting the therapeutic effect.
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Objective To investigate whether topical mucopolysaccharide polysulfate (MPS)cream can reduce the incidence of eczema and skin atrophy in patients with moderate-or low-risk infantile hemangioma after the treatment with topical beta-blockers or 595-nm pulsed dye laser(PDL), and to analyze factors influencing the occurrence of eczema and skin atrophy. Methods A total of 722 patients aged 0-1 years with moderate-or low-risk infantile hemangioma were enrolled from 5 Children' s Hospitals in China. According to the disease condition and therapy acceptability, these patients were divided into 6 groups to be treated with topical beta-blockers and MPS cream(blocker+MPS group), topical beta-blockers(blocker group), 595-nm PDL and topical MPS cream(PDL+MPS group), 595-nm PDL(PDL group), 595-nm PDL combined with topical beta-blockers and MPS cream(PDL+blocker+MPS group), and 595-nm PDL and topical beta-blockers(PDL+blocker group), respectively. All the externally applied agents were applied twice a day, and PDL was performed once every 4 weeks. Efficacy and adverse reactions were evaluated after 3-month treatment. Univariate and multivariate Logistic regression analyses were carried out to analyze factors influencing the incidence of eczema and skin atrophy in patients with infantile hemangioma after treatment, and chi-square test was carried out to compare efficacy among the groups. Results After 3-month treatment, multivariate Logistic regression analysis for comparing the blocker + MPS group with blocker group showed that the risk factor for eczema on the surface of hemangiomas was no topical treatment with MPS cream(P = 0.007, OR = 3.887, 95％ CI: 1.439- 10.493), while no correlations were observed between the occurrence of skin atrophy on the surface of hemangiomas and analyzed factors. Multivariate Logistic regression analysis for comparing the PDL + MPS group with PDL group showed that no topical treatment with MPS cream(P < 0.001, OR = 7.402, 95％ CI: 2.604- 21.042)and northern areas(P <0.001, OR=67.048, 95％CI:7.977-563.518)were risk factors for eczema on the surface of hemangiomas, and risk factors for skin atrophy on the surface of hemangiomas included no topical treatment with MPS cream(P=0.001, OR=9.371, 95 CI:2.590-33.900)and abundant blood supply of hemangiomas(P=0.036, OR=2.971, 95％CI:1.075-8.208). Multivariate Logistic regression analysis for comparing the PDL+ blocker + MPS group with PDL + blocker group showed that risk factors for eczema on the surface of hemangiomas were no topical treatment with MPS cream(P=0.005, OR=3.426, 95％CI:1.446-8.119) and northern areas(P<0.001, OR=31.704, 95％CI:6.924-145.158), and risk factors for skin atrophy on the surface of hemangiomas included no topical treatment with MPS cream(P<0.001, OR=6.011, 95％CI:2.558- 14.126) and southern areas (P = 0.022, OR = 3.021, 95％ CI: 1.177- 7.753). After 3-month treatment, the response rate was significantly higher in the PDL group than in the PDL+MPS group(χ2=4.531, P = 0.033), and significantly higher in the blocker group than in the blocker + MPS group (χ2 =4.344, P=0.037). There were no significant differences in the response rate or cure rate among the other groups(all P>0.05). Conclusion During the treatment of moderate-or low-risk infantile hemangioma with topical beta-blockers or 595-nm PDL, the combination with topical MPS cream can reduce the occurrence of eczema and skin atrophy without affecting the therapeutic effect.
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Objective To assess the clinical value of magnetic resonance imaging (MRI) in the diagnosis of deep vascular diseases in children with superficial vascular diseases.Methods From January 2012 to December 2016,105 children with superficial vascular diseases in the Department of Dermatology of Shanghai Children's Hospital underwent MRI examination,and the clinical and imaging data were analyzed.Results Of the 105 children,42 were male and 63 were female.Their ages ranged from 0.5 to 168 months with an average age of 21.6 months.As MRI showed,25 (23.8％) children had deep vascular diseases,which could not be seen with naked eyes.These deep vascular diseases included multisite deep capillary hemangiomas in 15 cases,cranial-facial hemangioma syndrome in 4,arteriovenous malformation in 3,persistent intracranial artery sinus in 2,and varicosity and bone hypertrophy syndrome in 1.Conclusion MRI is of great value to the diagnosis of deep vascular diseases in children with superficial vascular diseases.
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Objective To investigate the prevalence and progression process of atopic diseases in adolescents, and to assess their relationship with filaggrin(FLG)mutations. Methods Totally, 334 adolescents aged from 11 to 19 years in a middle school in shanghai were enrolled into this study. A clinical interview was carried out to determine the prevalence of atopic diseases (such as ichthyosis, atopic dermatitis (AD), asthma, rhinitis, etc)in these subjects. Peripheral blood samples were collected from 285 out of the 334 adolescents for screening for common FLG mutations, including 3321delA and K4671X. Five years later, these adolescents were followed up for reevaluation of clinical presentations of atopic diseases. Statistical analysis was carried out by the chi-square test with the SPSS 20.0 software. Results As the baseline survey showed, 19 (5.69%)of the 334 adolescents had AD, 14 (4.19%)had ichthyosis vulgaris, 36(10.78%)had allergic rhinitis, and 4(1.20%)had asthma. FLG mutations were observed in 24(8.42%) of the 285 adolescents. Five years later, 265 adoscents completed the follow-up, and 69 (20.66%)were lost to follow-up. Of the 265 adolescents reevaluated, 13(4.89%)had AD, 15(5.64%)had ichthyosis vulgaris, 27(10.15%)had allergic rhinitis, and 1 (0.38%)had asthma. By the time the second survey was performed, 6 out of the 19 patients initially diagnosed with AD had achieved complete regression, 13 had experienced a marked decrease in SCORing atopic dermatitis (SCORAD)score, and symptoms had disappeared in 9 of the 36 patients initially diagnosed with allergic rhinitis. The frequency of FLG mutations was 10.0%in patients with AD, 55.6%in those with ichthyosis, and 40.0%in those with both AD and ichthyosis, and the development of ichthyosis was associated with FLG mutations(P<0.001). Conclusions The frequency of common FLG mutations was 8.42%in these adolescents. FLG gene may be a semidominant gene associated with ichthyosis vulgaris, and multiple factors influence its expression.
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The natural course of atopic dermatitis (AD) has been a key subject with major concerns of physicians and patients.Tne pathogenesis of AD is complicated, which is associated with genetic, immunological,environmental and lifestyle factors, resulting in skin barrier impairment and immune system dysfunction.Therefore, there are individual differences in the age of onset, duration, aggravation, prognostic factors for remission and response to treatment.In recent years, with evidence of genetic research and a large amount of clinical retrospective studies,we have a more comprehensive understanding of the natural course of AD,which will help develop individualized treatment and evaluation strategies.