BACKGROUND Lichen planus(LP)with distribution of lesions along Blaschko’s lines is a rare entity,accounting for 0.24%-0.62%of all patients.Unilateral distribution of lesions in arm,leg,trunk,and waist is even less co...BACKGROUND Lichen planus(LP)with distribution of lesions along Blaschko’s lines is a rare entity,accounting for 0.24%-0.62%of all patients.Unilateral distribution of lesions in arm,leg,trunk,and waist is even less common.Approximately 10%of patients with LP manifest nail lesions.CASE SUMMARY A 20-year-old woman presented to our department with polygonal,purpuric,flattopped papules over the right arm,right leg,and right side of trunk and waist for the last 5 mo.The patient initially developed nail deformation in the left middle finger with no obvious cause,followed by development of blue-purple and red maculopapular rash with pruritus.During the disease course,the skin lesions aggravated and spread to several segments due to scratching.The lesions showed unilateral distribution along the Blaschko’s lines.The diagnosis of LP along Blaschko’s lines was established based on dermoscopy and skin biopsy.Her cutaneous lesions considerably improved after 4-wk treatment with intramuscular glucocorticoid,oral acitretin,topical glucocorticoid,and retinoids.CONCLUSION Cases of LP involving multiple segments of the body along the Blaschko’s lines with nail damage are rare.展开更多
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
基金Supported by National Natural Science Foundation of China,No.81803160.
文摘BACKGROUND Lichen planus(LP)with distribution of lesions along Blaschko’s lines is a rare entity,accounting for 0.24%-0.62%of all patients.Unilateral distribution of lesions in arm,leg,trunk,and waist is even less common.Approximately 10%of patients with LP manifest nail lesions.CASE SUMMARY A 20-year-old woman presented to our department with polygonal,purpuric,flattopped papules over the right arm,right leg,and right side of trunk and waist for the last 5 mo.The patient initially developed nail deformation in the left middle finger with no obvious cause,followed by development of blue-purple and red maculopapular rash with pruritus.During the disease course,the skin lesions aggravated and spread to several segments due to scratching.The lesions showed unilateral distribution along the Blaschko’s lines.The diagnosis of LP along Blaschko’s lines was established based on dermoscopy and skin biopsy.Her cutaneous lesions considerably improved after 4-wk treatment with intramuscular glucocorticoid,oral acitretin,topical glucocorticoid,and retinoids.CONCLUSION Cases of LP involving multiple segments of the body along the Blaschko’s lines with nail damage are rare.
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.
