Multiple porokeratomas (porokeratotic acanthoma) coexisting with disseminated superficial porokeratosis: Clinical,dermoscopic and pathological observations,and review of published work

Porokeratoma is a recently described solitary or multiple tumor-like acanthoma, sharing the histological feature of cornoid lamellae with porokeratosis. Whether porokeratoma is a variant of porokeratosis is controversial. We report a rare case of a 53-year-old Chinese woman who presented with multiple, symmetrical, hemispherical and verrucous plaques on her lower extremities that had been present for 20 years. The clinical manifestation resembled the fungal disease of chromoblastomycosis. The diagnosis of multiple porokeratoma coexisting with disseminated superficial porokeratosis was rendered according to the clinical, dermoscopic and pathological features. Oral acitretin (30 mg/day) and laser therapy were administrated. After 6 months of treatment, the number of plaques on her limbs was significantly reduced without recurrence. From this case, we speculate that porokeratoma is a rare and special variant of porokeratosis. In complex cases with multiple lesions, oral acitretin can be combined with surgery, cryotherapy and laser therapy.     

Acitretin exerted a greater influence on T‐helper (Th)1 and Th17 than on Th2 cells in treatment of psoriasis vulgaris

T‐helper (Th) cells, including Th1, Th2 and Th17 cells, may play an important role in the pathogenesis of psoriasis vulgaris (PV). Acitretin is an effective treatment for PV; however, its influence on Th cells during the treatment of PV is unclear. This study aimed to investigate the influence of acitretin on Th1, Th2 and Th17 cells in PV patients. PV patients (n = 30) received acitretin p.o. (20 mg/day) for 8 weeks. Sera and skin biopsies were obtained before and after treatment. Double‐labeled immunofluorescence was used to analyze T, Th1, Th2 and Th17 cells in skin lesions. Enzyme‐linked immunosorbent assay and western blot were used to analyze the expressions of interferon (IFN)‐γ, interleukin (IL)‐4 and IL‐17 in sera and skin lesions. The expressions of IFN‐γ mRNA, IL‐4 mRNA and IL‐17 mRNA in skin lesions were detected by in situ hybridization. Acitretin decreased the quantity of T, Th1 and Th17 cells in PV lesions, but had no significant influence on Th2 cells. Acitretin also decreased the expression of IFN‐γ and IL‐17 in serum and lesions. The expressions of IFN‐γ mRNA and IL‐17 mRNA decreased significantly after 8 weeks of therapy. However, acitretin had no significant influence on the expression of IL‐4 protein and mRNA. Acitretin can reverse Th1 and Th17 preponderance in PV patients to some degree. This may be due to the mechanism of acitretin on PV; however, Th2 cells were not affected by acitretin treatment.     

阿维A与氨甲蝶呤治疗重度斑块型银屑病的疗效

目的：观察阿维A与氨甲蝶呤治疗重度斑块型银屑病的疗效。方法：54例确诊为斑块型银屑病的患者被分为阿维A组(32例,口服阿维A30 mg,每日1次)和氨甲蝶呤组(22例,口服氨甲蝶呤15 mg,每周1次),均外用卡泊三醇软膏和硼酸软膏,疗程8周。以银屑病面积和严重度指数(psoriasis area and severity index,PASI)作为观察指标,比较两种药物的疗效。 结果：治疗后,阿维A组和氨甲蝶呤组PASI评分均较治疗前下降(P<0.001),PASI评分改善率和PASI评分有效率在两组间比较差异均无统计学意义(均P>0.05)。结论：阿维A或氨甲蝶呤治疗重度斑块型银屑病均有效,且疗效相当。     

Long‐term safety and efficacy of continuous acitretin monotherapy for three children with different severe hyperkeratotic disorders in China

Long‐term systemic treatment with acitretin for severe hyperkeratotic disorders is needed to maintain quality of life of afflicted patients, but treatment has been limited owing to its potential side‐effects including skeletal malformations, particularly for children during their growth and development. A retrospective investigation was conducted with three children afflicted with a severe hyperkeratotic disorder, namely Darier's disease, bullous ichthyosiform erythroderma or lamellar ichthyosis, who were continuously maintained on 0.2–0.3 mg/kg per day acitretin for more than 12 years after an initial period at a larger acitretin dose to bring each disease under control. The patients had good responses to acitretin treatment, which was assessed for safety, skeletal abnormalities, growth retardation and other potential side‐effects. Acitretin monotherapy was an effective treatment for these children, and maintenance doses were well tolerated with no skeletal or other observable side‐effects during the course of the study.     

Multiple eruptive squamous cell carcinoma in a patient with chronic plaque psoriasis on adalimumab

A 67‐year‐old man with chronic plaque psoriasis previously treated with psoralen plus PUVA, ciclosporin, methotrexate and acitretin developed eruptive squamous cell carcinoma after seven doses of adalimumab. We review the association of squamous cell carcinoma with immunosuppressive agents used for the treatment of chronic plaque psoriasis. Initiation of tumour necrosis factor (TNF)‐α inhibitors in a patient at high risk of non‐melanoma skin cancer may warrant chemoprophylaxis with acitretin.     

Treatment of lipoid proteinosis with acitretin in two patients from two unrelated Chinese families with novel nonsense mutations of the ECM1 gene

Lipoid proteinosis is a rare recessive genetic disorder caused by loss‐of‐function mutations to chromosome 1 at 1q21, the extracellular matrix protein 1 (ECM1) gene. Two children with lipoid proteinosis were reported from two unrelated Chinese families, both manifesting with a typical hoarse voice, white acne‐like atrophic lesions and scarring on the skin, and beaded papules around the eyelids. The diagnosis had been confirmed by laboratory tests, skin biopsy and laryngoscope examination. Genomic DNA sequencing was performed for both children and their family members. The two children were treated with acitretin for 6 months and followed up for 1 year. Genomic DNA sequencing of the ECM1 gene showed a novel homozygous nonsense mutation of C1522>T (p.R508X) at exon 10 in one patient, and a novel compound heterozygote for a nonsense/frame‐shift combination of mutations of R281X/1596delG at exons 7 and 10 in the other patient. The symptom of hoarse voice was improved by 6‐month treatment with acitretin, while there was no improvement in the skin lesions. These results demonstrated that acitretin treatment may have efficacy for some of patients with lipoid proteinosis, with superior effect on laryngeal symptoms than skin lesions. However, the conclusive therapeutic effect and underlying mechanisms remain to be further investigated.     

阿维A对中重度寻常型银屑病患者外周血MCP-4表达水平的影响

目的:检测中重度寻常型银屑病患者阿维A治疗前后血清MCP-4水平的变化。方法:26例中重度寻常型银屑病患者口服阿维A治疗8周。采用ELISA法检测正常对照和银屑病患者治疗前后血清MCP-4的表达水平。结果:寻常型银屑病外周血MCP-4为196.64±35.21 pg/mL,明显高于正常对照组(42.83±9.68 pg/mL),阿维A治疗后血清MCP-4为107.23±21.57 pg/mL,与治疗前相比明显降低(P0.01),但仍高于正常对照组(P0.01)。结论:阿维A可能通过调节外周血MCP-4表达水平发挥治疗银屑病作用。     

阿维A口服联合NB-UVB照射治疗寻常型银屑病疗效观察

目的观察阿维A胶囊口服联合NB-UVB照射治疗中、重度寻常型银屑病的疗效及安全性。方法对32例中、重度患者采用阿维A胶囊口服配合NB-UVB每周二次照射,共治疗8周,用PASI积分评价疗效,并记录不良反应。结果治疗开始1～2周后出现疗效,随着治疗时间的延长,有效率逐渐提高,治疗结束时痊愈率达到87.50%,有效率达96.88%。观察期间未见严重不良反应。结论阿维A胶囊口服联合NB-UVB照射治疗中、重度寻常型银屑病具有良好疗效和安全性。