Effects of short-term oral acitretin therapy on peripheral nerve function: a prospective neurological and neurophysiological study

The aim of the present prospective study was to substantiate possible side effects of short-term oral acitretin therapy (1 mg/kg/day) on peripheral nerve function of 13 patients with severe keratinization disorders. Clinical neurological examination before and 1 and 3 months after onset of treatment was unremarkable in all patients; however, a significant alteration of one or more neurophysiological, predominantly sensory, parameters was demonstrated in 3 out of 13 patients (23%) after 1 month and in 9 out of 13 (69%) after 3 months of oral acitretin therapy. These findings indicate that oral acitretin might be capable of causing a dysfunction of predominantly sensory nerve fibres in some individuals. Although in the investigated patients this dysfunction remained subclinical, it seems reasonable to suggest that neurological and neurophysiological evaluation of peripheral nerves should be added to the list of investigations that are routinely performed in patients receiving oral acitretin.     

重症板层状鱼鳞病一例

患者,女,41岁。全身黑褐色片状鳞屑40余年,伴瘙痒3天。结合临床及组织病理检查,诊断符合板层状鱼鳞病。口服阿维A、药物封包治疗4周后病情好转。     

Epidermodysplasia verruciformis: association with isolated IgM deficiency and response to treatment with acitretin

We describe a 25-year-old woman, who had extensive, large viral warts consistent with epidermodysplasia verruciformis (EV) since she was 6-year-old. Laboratory studies revealed an isolated IgM-deficiency, but the patient demonstrated no other abnormalities. She was treated with oral acitretin (0.5-1 mg/kg/day) for six months and her skin lesions improved slightly. However, after discontinuing the treatment, the lesions came back but she declined further treatment.     

Acitretin-induced subungual hemorrhage

A 20-year-old woman with a 2-year history of histologically confirmed palmoplantar keratoderma due to psoriasis, resistant to several topical agents, was admitted to the Department of Dermatology, Uludag University, Bursa, Turkey. Therapy with oral acitretin (0.5 mg/kg/day, 35 mg/day) was initiated. A month after starting acitretin treatment, she noted slight reddening of the second left fingernail. Clinical examination revealed red-brown discoloration of the second fingernail associated with subungual hemorrhage involving the proximal nail bed (lunula region) (Fig. 1). The nail change was asymptomatic. The patient complained only of discoloration underneath the nail plate. No abnormalities were detected on the skin, mucous membranes, or toenails/other fingernails. The patient denied exposure to microtrauma or any other drugs. The erythrocyte sedimentation rate, full blood cell count, electrolytes, renal and hepatic tests, and serum lipids were normal. Coagulation tests, including blood clotting time, international normalized ratio, activated partial thromboplastin time, thrombin time, platelet number, and function tests, were within normal levels. Treatment with acitretin was discontinued, and the nail change resolved completely after 3 weeks. A similar episode of subungual hemorrhage recurred, however, within 48 h after re-challenge with a lower dose of acitretin (25 mg/day). The drug was definitively stopped and the eruption faded again within a week. An objective causality assessment suggests that subungual hemorrhage was probably related to acitretin in this patient.     

Blastomycosis-like pyoderma: successful treatment with low-dose acitretin

Blastomycosis-like pyoderma typically presents as verrucous plaque(s) with a purulent discharge through multiple sinuses. Two patients with blastomycosis-like pyoderma developing on significantly sun-damaged skin are presented: an 84-year-old man with multiple painful nodules and plaques, and a 92-year-old woman with a solitary plaque on the left calf. Histopathology showed pseudoepitheliomatous hyperplasia with dermal foci of suppuration. Staphylococcus aureus was isolated from a biopsy specimen in the first patient, whereas the latter patient had Prevotella and Corynebacterium species. Investigations for fungi and mycobacteria yielded negative results. These microbiological findings further supported the clinicopathological diagnosis. Both patients failed to respond to oral doxycycline despite the in vitro susceptibility of the organisms. Treatment with low-dose oral acitretin was successful in both patients after 3-4 months.     

Treatment of verrucous carcinoma of vulva with acitretin

We describe a 60-year-old patient with verrucous carcinoma of the vulva, which recurred 6 years after simple vulvectomy and radiotherapy. Treatment with acitretin led to significant improvement and ongoing disease control has been achieved with low-dose maintenance therapy of 10 mg acitretin daily.     

Spontaneous resolution of facial papular mucinosis in a transplant patient

A 37-year-old male developed facial papules 6 months post renal–pancreatic transplant. Histological findings were consistent with localized papular mucinosis; electrophoresis showed no paraprotein. A trial of erbium and aura lasers, at ablative doses, produced no improvement. Independent treatments with oral doxycycline, itraconazole, acitretin, and isotretinoin also had no effect. The facial papules improved spontaneously, 2 years after first presentation and 8 months after treatment ceased. The patient remains clear of lesions.     

Acitretin for lichen amyloidosus

We present two cases of lichen amyloidosus treated with retinoids. A 57-year-old Vietnamese woman has had extensive generalized recalcitrant lichen amyloidosus for 23 years. Treatment with oral etretinate (25 mg/day) for 3 years, and later oral acitretin (10 mg/day) for the past 10 years, has controlled the pruritus and flattened the hyperkeratotic papules. Whenever the acitretin was ceased her symptoms flared within weeks. On each occasion reintroduction of acitretin was effective within 1-2 months. The second case is that of a 51-year-old Australian Aboriginal woman who had a 2-year history of lichen amyloidosus affecting her lower legs. A 2-month course of oral acitretin (25 mg b.d.) produced a marked improvement in both the pruritus and hyperkeratotic papules. She was then lost to follow up for 2 years, during which time her symptoms recurred.     

Generalized linear porokeratosis: a rare entity with excellent response to acitretin

Linear porokeratosis is a rare disorder of keratinization that usually presents at birth. We report a 17-year-old male with generalized linear porokeratosis, a very rare variant of porokeratosis, with extensive involvement of the trunk and extremities along with nail and genital involvement. The patient was treated with oral acitretin with excellent clinical response.     

Monilethrix

Monilethrix is a rare hereditary disorder that affects the hair and is characterized by shaft anomaly. There is no known treatment that successfully cures the condition. In this report we present a case of monilethrix in a 7-year-old girl treated with oral acitretin. A very good clinical and cosmetic result was obtained while treatment was continued. However, clinical symptoms recurred after discontinuation of acitretin therapy.     

Lichen ruber ulcerosus plantae

A 66-year-old patient suffering from painful ulcerative lichen planus of the feet is presented. Various treatments (topical and oral corticosteroids, irradiation and a 6-month therapy with acitretin) were tried without success. Skin grafting in the affected areas was helpful and resulted with an acceptable and painless state.     

维A酸治疗银屑病致维A酸综合征一例

报道维A酸治疗银屑病致维A酸综合征1例。患者男,32岁。有寻常性银屑病病史10余年。患者10余年来,头皮、躯干、四肢反复发红色斑丘疹、斑块,表面覆有多层银白色鳞屑,伴瘙痒,确诊为银屑病,长期口服维生素类、中成药及外用药物治疗,皮损缓解与加重交替。半年前因皮疹加重,给予阿维A（方希）胶囊10 mg每日2次共20 d,后增至10 mg每日3次共30 d,皮疹好转后,减至10 mg每日2次巩固治疗,连续用药半年,皮疹基本消退。就诊前1周改为口服维胺酯（三蕊）胶囊50 mg每日2次,3 d后皮疹加重,炎症反应明显,随后皮疹迅速泛发全身,融合成大片状,水肿明显,大量脱屑,伴高热,体温达39 ℃以上,并伴低血压、呼吸困难、肺水肿、胸腔和心包积液、肾功能异常及血白细胞明显升高。诊断为维A酸综合征。经糖皮质激素及对症支持治疗,症状均改善。维A酸综合征发生急骤,病情凶险,需及时诊断、并积极采用糖皮质激素及对症支持治疗。     

Violaceous papules on the palms and soles

BACKGROUND: Lichen planus (LP) is a condition with many clinical variants that can be quite varied in their presentation. OBJECTIVE: We report a case of a 60-year-old woman who presented with a very unusual palmoplantar eruption characterized by violaceous pustule-like papules that was subsequently diagnosed as LP. METHODS: The above case is reported, along with biopsy results. The patient was subsequently treated with a combination of topical corticosteroids and oral acitretin, and her clinical course was followed. RESULTS: The eruption responded dramatically to our combination treatment and resolved by the 2-month follow-up. CONCLUSION: Palmoplantar LP is a rare variant of LP that bears little resemblance to its classic LP. This condition responds well to a combination of potent topical corticosteroids and oral acitretin.     

阿维A联合阿达帕林凝胶治疗融合性网状乳头瘤病2例

目的观察阿维A联合阿达帕林凝胶治疗融合性网状乳头瘤病2例的疗效。方法口服阿维A30mg/d联合阿达帕林凝胶外用,皮损明显消退后,停用阿维A,继续外用阿达帕林凝胶至皮损全部消退。结果分别于联合治疗3周和5周后皮损明显消退,总疗程2～4个月,口服阿维A疗程7～9周。结论联合疗法可缩短阿维A的疗程,减轻不良反应,复发程度较初诊时轻微,再次治疗仍有效。     

炎症性浅表播散性汗孔角化症一例

患者,男,64岁。全身褐色环形斑块2年,伴瘙痒2个月。组织病理示：表皮角化不全,角化过度,可见角化不全柱,其下方棘层散在角化不良细胞,基底层灶状液化变性,真皮见团块状淋巴细胞为主的炎细胞浸润。诊断：炎症性浅表播散性汗孔角化症。给予阿维A 30 mg/d口服治疗,皮损部分消退,目前随访中。     

β受体阻滞剂致扁平苔藓样药疹一例

患者,男,69岁,皮损表现为躯干、四肢密集或散在紫红色扁平斑丘疹40天,以四肢末端为著,伴瘙痒,近2年间断口服酒石酸美托洛尔片（β受体阻滞剂）。病理检查符合扁平苔藓样药疹诊断。停用酒石酸美托洛尔片,给予口服阿维A,30mg,日1次,共1个月,外用3%水杨酸软膏、0.1%他克莫司软膏,皮损消退,效果明显。     

表现为发疹性瘙痒性丘疹的汗孔角化病

报告1例表现为发疹性瘙痒性丘疹的汗孔角化病.患者男,50岁.臀部丘疹伴剧烈瘙痒7年,逐渐融合成斑块,丘疹突然增多并累及双下肢1年.皮损组织病理检查符合汗孔角化病.给予角质剥脱剂外用及阿维A口服治疗4周,皮损暂无明显改善.     

Wong型无肌病性皮肌炎一例

患者,女,72岁。头面颈部红斑、丘疹伴瘙痒16年,日晒后加重。实验室检查示：抗TIF1-γ抗体阳性。额部皮肤组织病理表现为表皮角化过度,局灶性角化不全和界面皮炎改变。结合患者典型皮肌炎皮肤表现和额部毛发红糠疹样皮疹,诊断为Wong型无肌病性皮肌炎。给予阿维A、海棠合剂、1%吡美莫司乳膏等治疗,病情逐渐好转。     

Acitretin for erythrokeratodermia variabilis in a 9-year-old girl

Erythrokeratodermia variabilis (EKV) is a rare genodermatosis with a unique phenotype. Treatment with oral synthetic retinoids is well documented in adults, but not in children. We report a 9-year-old girl with EKV who cleared rapidly following treatment with oral acitretin.     

豪猪状鱼鳞病一例诊断与治疗观察

患儿男,10岁.因全身出现红色角化性扁平丘疹及褐黄色钉状突起性角化性斑块来我所就诊.皮肤科检查:头面部、躯干、四肢大片红色角化斑块基础上疣状、钉样突起,表面结褐黄色油腻痂,皮损多融合,如"豪猪"样外观.背部皮损组织病理学检查示角化过度,局部柱状角化不全,棘层增生肥厚,角质层偶见棘突松解性角化不良细胞,真皮乳头不规则向上增生,浅层血管周围淋巴组织细胞浸润,根据病理结果诊断为豪猪状鱼鳞病.给予口服阿维A胶囊及外用0.1%的维A酸乳膏持续治疗8年,起始剂量0.5mg·kg-1·d-1,最大剂量1 mg·kg-1·d-1.治疗期间定期监测患儿肝肾功能,血脂及身高、体质量,不良反应除皮肤黏膜干燥外,其他无异常.