Comparison of efficacy and safety of biologics versus methotrexate in the treatment of severe pediatric plaque psoriasis / 中华皮肤科杂志

Objective:To compare the efficacy and safety of biologics versus methotrexate in the treatment of severe pediatric plaque psoriasis.Methods:A retrospective matched case-control study was carried out. Twenty children with severe plaque psoriasis from Beijing Children′s Hospital, Capital Medical University from June 2016 to November 2021 were included in this study, and the patients treated with biologics (adalimumab or secukinumab) were matched with those treated with methotrexate at a ratio of 1∶1 according to the psoriasis area and severity index (PASI) score and age. PASI, physician′s global assessment (PGA) , and body surface area (BSA) scores were assessed at weeks 4, 8 and 12 after the start of treatment, and adverse drug reactions were recorded. Statistical analysis was mainly carried out by using Mann-Whitney U test, Fisher′s exact test and generalized estimating equations. Results:At weeks 4 and 8, the proportions of patients achieving PASI75 and PASI90 were significantly higher in the biologics group (PASI75: 7/10, 10/10, PASI90: 5/10, 9/10, respectively) than in the methotrexate group (PASI75: 1/10, 5/10, PASI90: 0, 1/10, respectively; all P < 0.05) , while there was no significant difference between the biologics group and methotrexate group at week 12 (PASI75: 10/10 vs. 8/10, PASI90: 9/10 vs. 4/10, both P > 0.05) . There were no significant differences in the PASI, BSA or PGA scores between the two groups at baseline (all P > 0.05) , while the biologics group showed significantly decreased PASI and BSA scores at weeks 4, 8 and 12, and significantly decreased PGA score at week 8 compared with the methotrexate group (PASI: Z = 2.50, 3.56, 2.63, respectively; BSA: Z = 2.87, 3.57, 2.40, respectively; PGA: Z = 2.81; all P<0.05) . Analysis of changes over time showed that the PASI, PGA and BSA scores in the biologics group significantly decreased at weeks 4, 8 and 12 compared with those at baseline (all P<0.01) ; the PASI and PGA scores significantly decreased at weeks 8 and 12 compared with the corresponding scores at week 4 (all P<0.05) ; however, there were no significant differences in the PASI, PGA or BSA scores between week 12 and 8 (all P>0.05) . In the methotrexate group, the PASI, PGA and BSA scores at weeks 4, 8 and 12 were all significantly lower than the corresponding scores at the previous adjacent time points (all P<0.05) . There was no significant difference in the incidence of adverse reactions between the two groups ( P = 0.650) , and no serious adverse reactions occurred in either group. The main adverse reaction was infection in the biologics group, while infection and elevation of transaminase levels were common in the methotrexate group. Conclusion:Biologics and methotrexate were both effective and safe for the treatment of severe pediatricplaque psoriasis, and biologics facilitated rapider achievement of PASI75 and PASI90 compared with methotrexate.

Progress in the Treatment of Topical Rapamycin Preparations / 罕见病研究

Rapamycin is a mammalian target of rapamycin(mTOR) receptor inhibitor. Advances in the understanding of the mTOR signaling pathway and its downstream effects on tumorigenesis and vascular proliferation have broadened the clinical applications of mTOR receptor inhibitors in treating many challenging diseases. Rapamycin is used orally for the treatment of kidney transplantation, lymphatic leiomyomatosis of lung, tuberous sclerosis complex(TSC), and etc. But systemic therapy using the rapamycin has significant side effects. To mitigate the side effects of systemic rapamycin for dermatologic applications, clinicians have used topical therapy. In recent years, research publications on the topical rapamycin in the treatment of a variety of diseases have increased, as on such diseases of facial angiofibroma of tuberous sclerosis complex, lymphatic malformation, Kaposi hemangioendothelioma, tufted angiomas, and etc. Topical rapamycin can be used as an effective long-term therapy while avoiding systemic side effects, providing a new treatment method for dermatologists. This paper discusses the progress in the treatment of topical rapamycin preparations.

Effect of oral acitretin on the height and bone development of children: a retrospective analysis of 106 cases / 中华皮肤科杂志

Objective:To evaluate the effect of oral acitretin on the height and bone development of children.Methods:Clinical and imaging data were collected from 106 children receiving oral acitretin for at least 1 month in Department of Dermatology, Beijing Children′s Hospital from March 2007 to January 2021, and retrospectively analyzed. The main outcome measures were height and near-adult height. Multivariate logistic regression analysis was carried out to investigate relevant factors for short stature in children, and non-inferiority test was used to analyze the proximity of the actual height to target height of children who had reached near-adult height. The secondary outcome measures were bone age and epiphyseal closure. Wilcoxon signed-rank test was used to analyze differences in the value of bone age minus chronological age between the baseline and last follow-up, and the premature closure of epiphysis was also evaluated.Results:Among the 106 children, 62 were males and 44 were females; 84 were diagnosed with pustular psoriasis, 10 with psoriasis vulgaris, 11 with pityriasis rubra pilaris, and 1 with lupus miliaris disseminatus faciei. These children received oral acitretin at doses of <1 mg·kg -1·d -1 for 1 - 90 months. Among the 96 children aged under 18 years, 91 (94.8%) were of normal stature, and 5 (5.2%) were short in stature; among the 83 children receiving acitretin monotherapy, 81 (97.6%) were of normal stature, and 2 (2.4%) of short stature. Binary logistic regression analysis showed that the risk of short stature caused by acitretin combined with glucocorticoid therapy was 76.57 times higher than that of acitretin monotherapy ( OR = 77.57, 95% CI: 2.20 - 2 738.82, P = 0.017) , while the type of disease, gender, age at onset, age at initial treatment with acitretin, course of treatment, and average daily dose of acitretin did not significantly affect the stature of children ( P = 0.988, 0.214, 0.087, 0.078, 0.066, 0.350, respectively) . At the last follow-up visit, 13 children who had reached near-adult height were of normal stature, and the non-inferiority test showed that their near-adult height was not inferior to the target height (Satterthwaite = 0.23, P = 0.030) . Bone age was evaluated in 45 children at baseline and last follow-up visit, there was no significant difference in the value of bone age minus chronological age between the baseline and last follow-up ( Z = -0.85, P = 0.250) , and no patients experienced premature closure of epiphysis before and after the treatment. Conclusion:This study preliminarily revealed that oral acitretin at doses of <1 mg·kg -1·d -1 for less than 90 months might not significantly affect the height and bone development of children.

Skin Rash as the First Manifestation of Pediatric Chronic Neutrophilic Leukemia / 罕见病研究

A 6-year-old girl presented with recurrent skin rash at the initial stage, recent joint pain, and neutrophilia was found during a routine blood test. After a multidisciplinary case discussion, she was diagnosed with chronic neutrophil leukemia, and the symptoms were relieved after hydroxyurea and luxolitinib treatment. She received the allogeneic hematopoietic stem cell transplantation subsequently. At present, she is in stable condition and under follow-up. Chronic neutrophil leukemia is a rare disease, which rarely occurs in children. It is more difficult to diagnose in patients with skin rash as the first manifestation. The diagnosis and treatment of this case reflects the important role of multidisciplinary cooperation in the diagnosis and treatment of difficult and rare diseases.

Clinical Features and Genetic Analysis of Autosomal Dominant and Recessive Hyperimmunoglobulin E Syndrome / 罕见病研究

  Objective  To summarize the clinical and genetic features of children with autosomal dominant and recessive hyperimmunoglobulin E syndrome (HIES).  Methods  HIES patients were studied at the dermatology department of Beijing Children's Hospital, Capital Medical University were collected, from January 2013 to December 2021, diagnosed by both clinical manifestation and genetic assessment. The general data were summarized, the clinical and genetic characteristics were analyzed, and the similarities and differences between autosomal dominant HIES (AD-HIES) and autosomal recessive HIES (AR-HIES) were compared.  Results  A total of 7 children with HIES were studied, including 3 cases of AD-HIES and 4 cases of AR-HIES. There were 4 males and 3 females. All children had recurrent eczema-like lesions, recurrent skin and pulmonary infections, and elevated serum IgE and eosinophil levels. The differences between AD-HIES and AR-HIES mainly include: the main cutaneous infection in 3 children with AD-HIES were bacterial infections (such as abscess and impetigo), while in 4 children with AR-HIES, cutaneous infections were mostly severe viral infection (such as verruca vulgaris and molluscum contagiosum). There were pulmonary parenchymal changes (such as pneumatoceles, cyst and atelectasis) in 3 children with AD-HIES, whilst there were no similar changes in the lungs of 4 children with AR-HIES; 75% of children with AR-HIES had allergic diseases (including asthma and food allergy), while there were no reports of allergic diseases in children with AD-HIES. As for manifestations outside of immune system, AD-HIES was more likely to appear facial dysmorphism(such a broad nasal bridge and a high-arched palate). Furthermore, the incidence of tumor in AR-HIES was higher than that in AD-HIES. AD-HIES was mainly caused by the mutation of STAT3 gene, and AR-HIES was mainly caused by the mutation of DOCK8 gene. We reported two new mutation sites of DOCK8 gene c.1798-2A > T and c.874G > A in two cases, respectively.  Conclusions  For children with clinical manifestations of recurrent eczema-like lesions, repeated infection and significant increase in serum IgE levels, HIES should be suspected, and genetic screening should be carried out to make definite diagnosis and classification, to achieve better long-term management and improve prognosis.

An investigation into the current situation of pediatric psoriasis based on the information systems from two children′s hospitals / 中华皮肤科杂志

Objective:To investigate clinical characteristics of pediatric psoriasis based on the information systems from two children′s hospitals.Methods:Clinical data on outpatients confirmly diagnosed with pediatric psoriasis were collected from information systems of Beijing Children′s Hospital affiliated to Capital Medical University and Children′s Hospital of Chongqing Medical University from January 1, 2015 to December 31, 2019, and a clinical and epidemiological investigation was conducted. Statistical analysis was carried out by using t test and chi-square test. Results:A total of 5 235 children with psoriasis were included, with the ratio of male to female being 1∶1.08. Their age at the clinic visit ( M [ Q1, Q3]) was 8.37 (6.48, 10.50) years, and the school-age children were the most common population; their age at onset was 7.57 (5.37, 9.82) years. Among the 5 235 children with psoriasis, there were 3 195 (60.82%) with psoriasis vulgaris, 281 (5.37%) with pustular psoriasis, 19 (0.36%) with erythrodermic psoriasis, and 1 (0.02%) with psoriatic arthritis. The trunk (87.76%, 1 097/1 250) was most frequently affected, followed by the limbs (87.68%, 1 096/1 250) , the scalp (62.56%, 782/1 250) , and the face and neck (35.76%, 477/1 250) . Among the 5 235 patients, 4 319 (82.50%) received topical treatments, 177 (3.38%) received systemic treatments, and 832 (15.89%) were treated with antibiotics. Among 3 497 children who received initial treatment regimens, the disease could be controlled in 3 423 (97.88%) without change in treatment regimens, while treatment regimens needed to be adjusted in 2.12%. Conclusions:In the two children′s hospitals, most children with psoriasis developed this condition and visited the clinic at school age, and the predominant clinical type was psoriasis vulgaris. Most skin lesions were extensive, and commonly occurred on the trunk and limbs. Scalp involvement was not uncommon. The condition could be controlled by topical treatments in most children with psoriasis, while a few patients needed systemic treatments.

Clinical efficacy and safety of topical sirolimus in the treatment of 19 cases of superficial vascular malformation in children / 中华皮肤科杂志

Objective:To investigate efficacy and safety of topical sirolimus cream in the treatment of superficial vascular malformation in children.Methods:A single-center prospective study was carried out. Children with superficial vascular malformation were enrolled into this study from Vascular Anomalies Clinic, Beijing Children′s Hospital, Capital Medical University from September 2019 to September 2020, and treated with 0.1% sirolimus cream. The efficacy was evaluated according to an international four-level classification system through imaging examination, dermoscopy and subjective evaluation, and adverse reactions during the treatment were monitored. Statistical analysis was carried out by t test, univariate analysis of variance or Fisher′s exact test. Results:A total of 19 children with superficial vascular malformations were enrolled, including 12 males and 7 females, aged 1 - 11.5 years. Fourteen children were diagnosed with vascular and lymphatic malformations, 3 with lymphatic malformations, and 2 with venous malformations. Sixteen children presented with lesions on the lower extremities, 8 were accompanied by pain, 2 presented with ulceration, and 6 had previous treatment history. After 6-month treatment, 3 patients achieved improvement of level Ⅰ, 4 of level Ⅱ, 4 of level Ⅲ, and 8 of level Ⅳ; 16 achieved improvement, and 12 achieved marked improvement. Six patients showed significantly decreased length, thickness and width of lesions after 6 months of treatment (1.83 ± 0.84 cm, 1.00 ± 0.55 cm, 2.25 ± 1.25 cm, respectively) compared with those before treatment (2.40 ± 0.95 cm, 1.35 ± 0.61 cm, 2.50 ± 1.34 cm, t = 5.22, 10.25, 3.73, respectively, all P < 0.05) . Gender, age, medical history and pain sensation did not significantly affect the therapeutic effect (all P > 0.05) , while diagnostic classification of vascular malformations significantly affected the therapeutic effect ( P = 0.008) . Among the 19 children, 2 had mild local burning sensation after the treatment. After 1- and 6-month treatment, the blood concentrations of sirolimus were both below 1.0 ng/ml. Conclusion:Topical sirolimus is effective and safe in the treatment of superficial vascular malformation in children.

Research progress of deficiency of interleukin-36 receptor antagonist and cutaneous sterile pustulosis / 中华实用儿科临床杂志

Cutaneous sterile pustulosis is categorized as a non-infectious and non-follicular impetigo, with a low prevalence and difficulty in the treatment.Deficiency of interleukin(IL)-36 receptor antagonist (DITRA) is an auto inflammatory disease featured by the decreased interleukin-36 receptor antagonist (IL-36Ra) activity caused by IL36 RN mutation.Functional or structural defects of IL-36Ra increase the secretion of inflammatory and pro-inflammatory factors by keratinocytes, macrophages and dendritic cells.Upregulation of IL-36 receptor agonists induce type 17 helper T lymphocytes to secrete IL-17, which is essential for the onset of multiple subtypes of aseptic pustulosis.Research on the relationship between DITRA and cutaneous sterile pustulosis is important for developing targeted therapies.

Ichthyosis follicularis, alopecia, and photophobia syndrome: a case report and mutation analysis / 中华皮肤科杂志

A 26-month-old male child was admitted for hair abnormality after birth. There was no hair growth on the scalp of the child after birth, rough skin and scattered rice-grain-sized follicular papules were observed all over the body, and erythema was seen in the perianal region. Fifteen days after birth, the patient was found to be photophobic. Skin examination showed diffusely distributed rice-grain-sized follicular papules all over the body, no growth of hair, eyelashes or eyebrows, and well-circumscribed perianal erythema with scaling at the edges. Genetic testing revealed a c.661T>A mutation in the MBTPS2 gene on the X chromosome of the child, which caused a substitution of phenylalanine by isoleucine at amino acid position 221 (p. Phe221Ile) . A heterozygous mutation at the same locus was identified in the patient′s mother. The patient was diagnosed with ichthyosis follicularis, alopecia, and photophobia syndrome.

Clinical analysis of 11 children with primary osteoma cutis / 中华皮肤科杂志

Objective To investigate clinical manifestations,pathological features and outcomes of primary osteoma cutis in children.Methods Eleven children with confirmed primary osteoma cutis diagnosed in Department of Dermatology,Beijing Children's Hospital between 2011 and 2018 were included into this study.The clinical manifestations,histopathological features,and outcomes were analyzed retrospectively.Results Among the 11 patients,7 were males and 4 were females.Primary osteoma cutis occurred within 22 months after birth in all the children,the median age of onset was 1 month,and the disease occurred during the first 6 months of life in 10 children.The skin lesions were characterized by skincolored or reddish indurated papules,plaques or nodules of varying size with slight epidermal atrophy.Three patients had local skin lesions,and 8 had multiple skin lesions.Serum calcium and parathyroid hormone levels were within normal limits in all the children,and no developmental deformity was observed at birth in any of the children.Histopathological examination revealed the formation of mature lamellar bone in the dermis in all the cases,which involved the subcutaneous adipose tissue in 5 cases.The skin lesions became stable 8-18 months after the occurrence in 10 patients,which was consistent with primary plaquelike osteoma cutis.Only 1 patient underwent a slowly progressive course,and the skin lesions involved subcutaneous deep tissues,leading to dyskinesia,which was consistent with progressive osseous heteroplasia.Conclusions Primary osteoma cutis in children mostly occurs in infancy,whose clinical manifestations are atrophic,indurated plaques or nodules,and its main pathological feature is the formation of mature lamellar bone.Long-term follow-up is needed,and attention should be paid to the occurrence of progressive osseous heteroplasia.

Vincristine combined with glucocorticoids for the treatment of 11 cases of infantile Kasabach-Merritt syndrome / 中华皮肤科杂志

Objective To explore effective therapy for Kasabach-Merritt syndrome (KMS).Methods Clinical data were collected from 11 infants with KMS in the Department of Dermatology,Beijing Children's Hospital affiliated to Capital Medical University between 2015 and 2017,and the efficacy of vincristine combined with glucocorticoids for the treatment of KMS was evaluated.Results Of the 11 patients,4 were male and 7 were female.Their average age at the first clinic visit was 87.91 ± 72.01 days (range,1-212 days).Most of the hemangiomas manifested as hard violaceous plaques,and skin purpura occurred around the hemangiomas in 5 patients.The initial blood platelet counts of the 11 patients ranged from 4 × 109/L to 32 × 109/L.All the patients were treated with oral prednisone at doses of (2-5) mg· kg 1· d1 combined with intravenous injection of vincristine at a dose of 0.05 mg/kg every week.Among 10 patients treated with glucocorticoids and vincristine,the blood platelet counts became normal after (1.8± 1.23) weeks of treatment,the fibrinogen levels returned to normal after (3.6 ± 1.26) weeks of treatment,and the hemangiomas started to become softer and smaller after (3.9 ± 0.74) weeks.The blood platelet count still did not return to normal in 1 patient after 5 weeks of vincristine treatment.After the treatment with venous embolization,the blood platelet count gradually became normal and remained stable.Conclusion Vincristine combined with glucocorticoids can control the development of hemangioma in infants with KMS,and improve the recovery of blood platelet count.

Ultrapulsed fractional CO2 laser for the treatment of nine cases of porokeratosis in children / 中华皮肤科杂志

Objective To investigate the efficacy and safety of ultrapulsed fractional CO2 laser in the treatment of porokeratosis in children.Methods Clinical data were collected from 9 children with porokeratosis in the Department of Dermatology of Beijing Children's Hospital from January 2014 to December 2015,and analyzed retrospectively.These patients were all treated with ultrapulsed fractional CO2 laser in a dynamic superficial stripping (Active FX) mode.The initial energies were 100,200 and 300 mJ/cm2,and the frequencies ranged from 100 to 300 Hz.Before and after the treatment,as well as during the follow-up,confocal laser scanning microscopy was used to evaluate the severity and recovery of skin lesions.Results Of the 9 patients,7 were male,and 2 were female.Their average age was 4.19 ± 3.97 years.After the treatment with ultrapulsed fractional CO2 laser,all of the patients were considered to be cured based on the clinical standard.Some adverse reactions such as erythema,edema and erosion occurred in the 9 patients immediately after the treatment,but all completely regressed within 3-7 days.At 3,6 and 12 months after the treatment,no scars or skin discoloration was observed.The average duration of follow-up was 1 year,and the longest duration of follow-up was 2 year and 4 months.No relapse occurred during the follow-up.Conclusion Ultrapulsed fractional CO2 laser is effective and safe for the treatment of porokeratosis in children.

Factors associated with the relapse of propranolol-treated infantile hemangioma / 中华皮肤科杂志

Objective To analyze factors associated with the relapse of propranolol-treated infantile hemangioma.Methods The clinical data were collected from 235 children with infantile hemangioma who had discontinued propranolol for 6 months,and retrospectively analyzed.Factors for the relapse of propranolol-treated infantile hemangioma were analyzed by univariate and multivariate unconditional logistic regression analyses.Results Of 235 patients followed for 6 months after drug withdrawal,66 (28.1％) were identified to have recurrence of infantile hemangioma,of whom,15 (22.7％) had severe recurrence.The risk of recurrence was significantly increased in patients taking propranolol at a daily dosage of 1.5 mg/kg compared with those taking propranolol at a daily dosage of 2 mg/kg (OR =3.566,95％ CI:1.306-9.739),in patients aged ＞ 8 weeks at the initial drug treatment compared with those aged ≤ 8 weeks at the initial drug treatment (OR =5.043,95％ CI:1.248-20.376),in patients with the course of medication ≤ 6 months compared with those with the course of medication ＞ 6 months (OR =17.661,95％ CI:4.899-63.665),as well as in patients aged ＜ 1 year at drug withdrawal compared with those aged ≥ 1 year at drug withdrawal (OR =6.089,95％ CI:1.835-20.204).Conclusion There are many risk factors associated with the relapse of propranolol-treated infantile hemangioma,so some measures aimed at these risk factors should be taken to reduce the recurrence rate.

Lupus erythematosus profundus of the scalp in five children / 中华皮肤科杂志

Objective To investigate clinical and pathological features of lupus erythematosus profundus (LEP)of the scalp in children.Methods A retrospective study was carried out on 5 children with LEP.The clinical and histopathological features,treatment and prognosis of LEP were analyzed.Results The 5 children with LEP included 2 boys and 3 girls with a median age at onset of 5 months (range,2-38 months) and a median clinical course of 15 months (range,4-72 months).Clinically,the patients presented with arc-shaped or circular purple atrophic plaques on the scalp complicated by alopecia.The occipitalia and tempora were the most commonly involved sites.Antinuclear antibodies (ANA) and extractable nuclear antigens (ENAs) were negative in all the patients.Main histopathological changes were hyaline degeneration of the fat,mucin deposition and local aggregation of lymphocytes in fat lobules.Of the 5 patients,2 were treated with oral prednisone (1.5-2 mg/kg/day),1 with oral hydroxychloroquine (5 mg/kg/day),1 with oral prednisone (1.5 mg/kg/day) combined with hydroxychloroquine (5 mg/kg/day),and another 1 with topical halometasone cream and 0.03％ tacrolimus ointment.Lesions were remissed after 2-3 months of treatment,and subsided with growth of new hairs after 6 months.No recurrence was observed during a 1.5-year follow-up.Conclusion Prednisone and hydroxychloroquine are markedly effective for LEP,and pediatric patients with LEP may be treated by topical highpotency glucocorticoids and calcineurin inhibitors.

Efficacy and safety of topical sirolimus in the treatment of facial angiofibromas in children with tuberous sclerosis complex / 中华皮肤科杂志

Objective To investigate the efficacy and safety of sirolimus 0.1% ointment in the treatment of facial angiofibromas in children with tuberous sclerosis complex. Methods Sirolimus 0.1% ointment was prepared. Twenty children with tuberous sclerosis complex who had facial angiofibromas were enrolled in this study. Facial angiofibromas were topically treated with the self?prepared sirolimus 0.1% ointment twice a day for 12 weeks. The facial angiofibroma severity index(FASI)was calculated, the degree of satisfaction with the treatment was evaluated, and adverse reactions were analyzed at weeks 4 and 12. Plasma sirolimus concentrations as well as blood biochemical and immunological parameters were measured, blood coagulation activity was evaluated, and routine blood tests as well as urine tests were performed at baseline and week 12. Results The FASI of patients significantly decreased at weeks 4(4.400 ± 1.284)and 12(2.975 ± 1.543)compared with that at baseline(5.750 ± 1.175, both P<0.000 1), and was significantly lower at week 12 than at week 4(P < 0.000 1). The efficacy index was 49.87% ± 22.08% at week 12, significantly higher than that at week 4(24.43%± 10.18%, t=7.338, P<0.01). The color, size and number of lesions significantly decreased in all the patients, and facial angiofibromas completely disappeared in 2 patients at week 12. At week 4, 10 parents were satisfied with the improvement of erythema, 3 parents with that of lesion volume, and 3 parents with that of lesion area. The degree of parent satisfaction increased at week 12 in all the cases. The blood concentration of sirolimus was lower than 1.0μg/L both before and after the treatment. No severe systemic or local adverse reactions were noted in these patients. Conclusion Sirolimus 0.1%ointment is markedly effective and safe for the treatment of facial angiofibromas in patients with tuberous sclerosis complex.

Primary cutaneous γδ-T cell lymphoma with hemophagocytic syndrome in a child / 中华皮肤科杂志

A 13-year-old girl presented with multiple recurrent cutaneous plaques for more than six months,which had been aggravated with intermittent fever for five months.No obvious systemic abnormality was found.Dermatological examination revealed multiple,non-ulcerative,painless,infiltrative,indurated,poorly marginated,purple subcutaneous plaques measuring 3-1 1 cm in diameter with slight squamation in bilateral buttocks and lower limbs.Laboratory investigations showed bicytopenia with the white blood cell count being (0.03-3.7) × 109/L and red blood cell count being (2.8-4.4) × 1012/L,a normal platelet count,hypofibrinogenemia (1.79 g/L) and low proportion of natural killer cells (4.6％).Bone marrow smear showed active proliferation of cells,decreased proportion of granulocytes,presence of a few indefinitely classified cells,and phagocytosis.Reticulocytes were easily seen in the bone marrow smear.Pathologically,no obvious abnormality was observed in the epidermis or dermis,while the subcutaneous adipose tissue,especially fat lobules and some interlobular septa,was extensively infiltrated by large-to medium-sized lymphoid cells with pleomorphic and twisted nuclei as well as a small amount of cytoplasm; necrosis and phagocytosis of nuclear debris and lymphocytes were visible.The atypical lymphoid cells stained positive for CD3,T-cell intracellular antigen-1,granzyme B and TCRγδ with partial loss of CD5 and CD7,but negative for CD56,CD4,CD8 and TCRαβ.No Epstein-Barr virus-encoded RNA (EBER) was detected by in situ hybridization.Based on these findings,a diagnosis of primary cutaneous γδ-T cell lymphoma with hemophagocytic syndrome was made.

Evaluation of skin barrier-related parameters in 116 healthy children / 中华皮肤科杂志

Objective To assess and compare physical and chemical properties of skin among different body areas of healthy children at different ages from Beijing.Methods A total of 116 healthy children were recruited from Beijing,China,and classified into four age groups.Tewameter TM300 was used to measure transepidermal water loss (TEWL) value,Corneometer CM825 to estimate stratum corneum hydration,and skinpH-Meter PH905 to determine skin surface pH,in the forehead,cheek and forearm.The Mexameter MX 18 probe to determine melanin index of the face.Analysis of variance was carried out to assess differences in these parameters among different measurement sites and age groups.Results TEWL value did not differ among different age groups or measurement sites (both P ＞ 0.05).The stratum corneum hydration value of forehead,cheek and forearm was 51.53 ± 15.70,39.88 ± 10.48 and 50.33 ± 17.54 respectively in the age group ＜ 1 year,49.95 ± 17.88,32.51 ± 12.09 and 36.10 ± 7.43 respectively in the age group 1-3 years,51.37 ± 10.60,31.65 ± 9.01 and 34.41 ± 8.21 respectively in the age group 4-6 years,49.74 ± 10.64,39.99 ± 50.43 and 29.35 ± 8.10respectively in the age group 7-12 years,with significant differences among different measurement sites (P ＜ 0.05) but not between different age groups (P ＞ 0.05).The cheek and forearm showed lower stratum corneum hydration value than the forehead.No statistical difference was observed in pH value at the same measurement sites between the age group ＜ 1 year (forehead 5.27 ± 0.60,cheek 6.12 ± 0.51 and forearm 5.48 ± 0.45),1-3years (forehead 4.68 ± 0.58,cheek 6.80 ± 0.55 and forearm 5.07 ± 0.58),4-6 years (forehead 4.58 ± 0.37,cheek 5.70 ± 0.48 and forearm 5.09 ± 0.49),and 7-12 years (forehead 4.87 ± 0.51,cheek 5.72 ± 0.49 and forearm 5.09 ± 0.51),but the cheek had significantly higher pH value than the forearm and forehead (both P ＜ 0.05).Melanin value on the face did not differ between different age groups (P ＞ 0.05).Conclusion Physical and chemical properties of skin vary with body sites in healthy children.

Efficacy and safety of propranolol in treating infantile hemangiomas / 中华皮肤科杂志

[Objective] To evaluate the clinical efficacy and safety of propranolol in treating infantile hemangiomas.[Methods] Ninety children with hemangioma collected from July 2010 to November 2011 were recruited in this study.Oral propranolol was given at a dose of 1.5-2.0 mg/kg per day,and the dose was adjusted according to the growth of body weight.Patients were revisited every month for the observation of appearance of hemangioma.The following parameters,including blood glucose,alanine transarninase,aspartate aminotransferase,urea nitrogen,creatinine,creatine kinase,heart rate,blood pressure,electrocardiogram and ultrasound image of hemangioma,were monitored before and after the administration of propranolol.[Results] A rapid halt in haemangioma proliferation was seen in 91.1％ (82/91) of the patients within 24-48 hours after the administration of popranolol.After 1-10 months of treatment,haemangioma shrunk by 0-25％ with a lightening of lesional color in 8.0％ (7/88) of the patients,by 26％-50％ with an obvious lightening of lesional color in 39.8％ (35/88),by 51％-75％ with a marked lightening of lesional color in 26.1％ (23/88),and 26.1％ (23/88)of the patients achieved a shrinkage of more than 75％ or fading of lesional color.The 7-8 months of treatment leaded to the best outcome,followed by 5-6 months,3-4 months,and 1-2 months,of treatrnent.No rebound was observed in patients who stopped the treatment at 10 months to l year and 4 months of age.Usually during early stage of the therapy,some of the patients suffered from reduced diastolic blood pressure,sleep disorder,loose stools,hypoglycemia,cold extremities,bronchial hyperreactivity,elevated alanine transaminase/aspartate aminotransferase or creatine kinase isoenzyme,most of which were tolerable and relieved with or without symptomatic treatment.[Conclusion]s Propranolol can suppress the growth and accelerate the regression of hemangiomas in proliferative phase,and also can promote the subsidence of hemangiomas in regressive phase.The side effects of propranolol are usually mild,but still need close monitoring.