Background Inflammatory linear verrucous epidermal nevus(ILVEN)is a rare type of epidermal nevus that occurs in infancy and develops along Blaschko’s lines.Due to its rarity and clinical/histological similarities wit...Background Inflammatory linear verrucous epidermal nevus(ILVEN)is a rare type of epidermal nevus that occurs in infancy and develops along Blaschko’s lines.Due to its rarity and clinical/histological similarities with other cutaneous disorders,correct diagnosis is sometimes difficult.In the present case,the whole exome sequencing of blood found no germline mutation,but the sequencing of the biopsy specimen revealed a novel potential pathogenic somatic mutation,C>T at rs200881715(p.R698X)in the cspp1 gene,which may provide new insight into the mechanism of ILVEN.Patient concerns A 10-year-old girl presented with linear,pruritic,erythematous,scaly,and exophytic verrucous skin lesions on her left thigh,buttock,and hand unilaterally along Blaschko’s lines from the third day after birth.Diagnosis Biopsy revealed psoriasiform acanthosis,papillary hyperplasia with orthohyperkeratosis,and parakeratosis of squamous epithelium,which are histological characteristics of ILVEN.Interventions Owing to the large lesion size and significant refractory response to previous treatments,multiple-stage surgical resections with a tissue expander were performed.Outcomes After surgical resection and defect reconstruction with a tissue expander,the outcome was satisfactory for the patient and her family.No recurrence was detected during the follow-up visits.Conclusion ILVEN is a rare type of cutaneous hyperplastic disease,and treatment is extremely challenging.Biopsy should be performed at the diagnostician’s discretion or if refractory to conservative treatments.Timely diagnosis and surgical intervention could result in satisfactory outcomes.展开更多
Background: Becker’ s naevus (BN) may represent a distressing cosmetic handicap and a challenging issue regarding treatment. Objectives: To compare clinical and histopathological findings of patients with BN treated ...Background: Becker’ s naevus (BN) may represent a distressing cosmetic handicap and a challenging issue regarding treatment. Objectives: To compare clinical and histopathological findings of patients with BN treated with two different lasers: an erbium: yttrium-aluminium-garnet (Er: YAG) system vs. a neodymium: YAG (Nd: YAG) laser. Patients and methods: In this prospective and comparative study we present the clinical and histopathological evolution during a 2-year follow-up of 22 patients with BN treated with only one pass of the Er: YAG laser (n=11) or three treatment sessions with the Q-switched Nd: YAG system (n=11). Results: Clinical evaluation 2 years after treatment with the Er: YAG laser showed complete clearance (100% ) in 54% of the patients (n=6) and clearance of>50% in 100% of the subjects. In relation to Nd: YAG laser treatments our results echo those of other authors. Numerous sessions are necessary to get an acceptable clinical clearance rate. Only one patient showed marked clearance(51-99% )after three treatment sessions. Moderate (26- 50% ) and mild (1-25% ) clearance was observed in 45- 5% (n=5) and 27-3% (n=3) of the patients. Conclusions: Both Er: YAG and Nd: YAG are safe tools to treat BN. However, in terms of pigment removal, one pass with Er: YAG is a superior technique to three treatment sessions with the Nd: YAG.展开更多
Background: Although vitiligo shows a similar clinical picture to other disorders in which patients exhibit depigmented spots, there are few reports on the differentiation of these conditions from each other. Purpose:...Background: Although vitiligo shows a similar clinical picture to other disorders in which patients exhibit depigmented spots, there are few reports on the differentiation of these conditions from each other. Purpose: We aimed to investigate the differences and similarities between vitiligo and nevus depigmentosus in children based on the clinical course and characteristics. Materials and Methods: We conducted an examination of the disease type, treatment, progress, and differentiation of 85 cases of vitiligo and nevus depigmentosus that developed before 15 years of age among patients who visited the Osaka University Department of Dermatology with a chief complaint of white patches from January 2010 to March 2017. Results: The median age at the onset of symptoms of 79 patients with vitiligo was 4.5 years and while that of 6 patients with nevus depigmentosus was 0.8 years, indicating that the onset of nevus depigmentosus occurred earlier. While vitiligo often developed in the head and neck regions, with a tendency for depigmented spots in multiple regions, nevus depigmentosus did not show this tendency in these regions. Upon performing Wood’s lamp observation in 38 cases of vitiligo, 35 cases were found to be fluorescent chalky white and in 3 cases histologically diagnosed as vitiligo, no clear fluorescent color was observed. All 6 patients diagnosed with nevus depigmentosus exhibited a non-fluorescent off-white color under a Wood’s lamp. Conclusions: This study indicates that non-invasive observation using a Wood’s lamp is useful in the differential diagnosis of pediatric leukoderma, in addition to the subsequent clinical course, such as the presence or absence of lesional repigmentation and/or enlargement.展开更多
An epidermal nevus(EN)is a common disease of various types,including verrucous EN,nevus sebaceous,inflammatory linear verrucous EN,Becker nevus,smooth muscle hamartoma,and nevus comedonicus.EN syndrome is a serious an...An epidermal nevus(EN)is a common disease of various types,including verrucous EN,nevus sebaceous,inflammatory linear verrucous EN,Becker nevus,smooth muscle hamartoma,and nevus comedonicus.EN syndrome is a serious and difficult-to-treat syndrome that is complicated by two or more abnormal organs or systems.To date,the mechanism of an EN is still unclear,and the therapies remain unsatisfactory.The most frequently used treatments are surgery and laser therapy.Several drugs have also been tested.An EN requires more attention,and further research is needed to provide appropriate and effective treatments for each patient.展开更多
基金This work was supported by grants from the Youth Doctor Collaborative Innovation Team Project(QC201803)of Shanghai Ninth People’s Hospital,Shanghai Jiao Tong University School of Medicine,Shanghai Youth Top-Notch Talent Program(201809004)Chenguang Program supported by the Shanghai Education Development Foundation and the Shanghai Municipal Education Commission(19CG18)+2 种基金the Science and Technology Commission of Shanghai Municipality(19JC1413)Shanghai Rising Star Program(20QA1405600)innovative research team of high-level local universities in Shanghai(SSMU-ZDCX20180700).
文摘Background Inflammatory linear verrucous epidermal nevus(ILVEN)is a rare type of epidermal nevus that occurs in infancy and develops along Blaschko’s lines.Due to its rarity and clinical/histological similarities with other cutaneous disorders,correct diagnosis is sometimes difficult.In the present case,the whole exome sequencing of blood found no germline mutation,but the sequencing of the biopsy specimen revealed a novel potential pathogenic somatic mutation,C>T at rs200881715(p.R698X)in the cspp1 gene,which may provide new insight into the mechanism of ILVEN.Patient concerns A 10-year-old girl presented with linear,pruritic,erythematous,scaly,and exophytic verrucous skin lesions on her left thigh,buttock,and hand unilaterally along Blaschko’s lines from the third day after birth.Diagnosis Biopsy revealed psoriasiform acanthosis,papillary hyperplasia with orthohyperkeratosis,and parakeratosis of squamous epithelium,which are histological characteristics of ILVEN.Interventions Owing to the large lesion size and significant refractory response to previous treatments,multiple-stage surgical resections with a tissue expander were performed.Outcomes After surgical resection and defect reconstruction with a tissue expander,the outcome was satisfactory for the patient and her family.No recurrence was detected during the follow-up visits.Conclusion ILVEN is a rare type of cutaneous hyperplastic disease,and treatment is extremely challenging.Biopsy should be performed at the diagnostician’s discretion or if refractory to conservative treatments.Timely diagnosis and surgical intervention could result in satisfactory outcomes.
文摘Background: Becker’ s naevus (BN) may represent a distressing cosmetic handicap and a challenging issue regarding treatment. Objectives: To compare clinical and histopathological findings of patients with BN treated with two different lasers: an erbium: yttrium-aluminium-garnet (Er: YAG) system vs. a neodymium: YAG (Nd: YAG) laser. Patients and methods: In this prospective and comparative study we present the clinical and histopathological evolution during a 2-year follow-up of 22 patients with BN treated with only one pass of the Er: YAG laser (n=11) or three treatment sessions with the Q-switched Nd: YAG system (n=11). Results: Clinical evaluation 2 years after treatment with the Er: YAG laser showed complete clearance (100% ) in 54% of the patients (n=6) and clearance of>50% in 100% of the subjects. In relation to Nd: YAG laser treatments our results echo those of other authors. Numerous sessions are necessary to get an acceptable clinical clearance rate. Only one patient showed marked clearance(51-99% )after three treatment sessions. Moderate (26- 50% ) and mild (1-25% ) clearance was observed in 45- 5% (n=5) and 27-3% (n=3) of the patients. Conclusions: Both Er: YAG and Nd: YAG are safe tools to treat BN. However, in terms of pigment removal, one pass with Er: YAG is a superior technique to three treatment sessions with the Nd: YAG.
文摘Background: Although vitiligo shows a similar clinical picture to other disorders in which patients exhibit depigmented spots, there are few reports on the differentiation of these conditions from each other. Purpose: We aimed to investigate the differences and similarities between vitiligo and nevus depigmentosus in children based on the clinical course and characteristics. Materials and Methods: We conducted an examination of the disease type, treatment, progress, and differentiation of 85 cases of vitiligo and nevus depigmentosus that developed before 15 years of age among patients who visited the Osaka University Department of Dermatology with a chief complaint of white patches from January 2010 to March 2017. Results: The median age at the onset of symptoms of 79 patients with vitiligo was 4.5 years and while that of 6 patients with nevus depigmentosus was 0.8 years, indicating that the onset of nevus depigmentosus occurred earlier. While vitiligo often developed in the head and neck regions, with a tendency for depigmented spots in multiple regions, nevus depigmentosus did not show this tendency in these regions. Upon performing Wood’s lamp observation in 38 cases of vitiligo, 35 cases were found to be fluorescent chalky white and in 3 cases histologically diagnosed as vitiligo, no clear fluorescent color was observed. All 6 patients diagnosed with nevus depigmentosus exhibited a non-fluorescent off-white color under a Wood’s lamp. Conclusions: This study indicates that non-invasive observation using a Wood’s lamp is useful in the differential diagnosis of pediatric leukoderma, in addition to the subsequent clinical course, such as the presence or absence of lesional repigmentation and/or enlargement.
文摘An epidermal nevus(EN)is a common disease of various types,including verrucous EN,nevus sebaceous,inflammatory linear verrucous EN,Becker nevus,smooth muscle hamartoma,and nevus comedonicus.EN syndrome is a serious and difficult-to-treat syndrome that is complicated by two or more abnormal organs or systems.To date,the mechanism of an EN is still unclear,and the therapies remain unsatisfactory.The most frequently used treatments are surgery and laser therapy.Several drugs have also been tested.An EN requires more attention,and further research is needed to provide appropriate and effective treatments for each patient.
