水源性肢端角化病

水源性肢端角化病是一种新发现的手足角化性疾病.临床表现为接触水数分钟后手和(或)足部突现半透明至白色的扁平丘疹或斑块,伴或不伴瘙痒、胀痛、烧灼或紧绷感,局部干燥后不久皮损消失或者改善.组织病理表现无特异性.其病因和发病机制尚不明确,缺乏特异的治疗方法.     

水源性肢端角化病1例

患儿男,5岁.双手足角化性丘疹及斑块近4年.患儿1岁始无明显诱因双手掌及手指和足跖出现角化性丘疹及斑块,接触水数分钟后皮损呈灰白色或乳白色,伴轻微胀痛.口角皮肤常浸渍发白.离水后半小时内皮损恢复原状,症状消失.平素手足多汗,随年龄增长,皮损逐渐扩大.患儿既往体健,无长期服药史,非特应性体质,无手足外伤及毛发、牙齿及甲异常.父母非近亲结婚,无家族史.诊断:水源性肢端角化病.     

水源性肢端角化病

患者男,32岁。主诉:双手浸水后出现丘疹、肿胀2年。现病史:患者2年前开始,双手接触水数分钟后出现白色丘疹、斑块,伴紧绷感。脱离水源,皮肤干燥后症状可完全消退。自发病以来未诊治。     

水源性肢端角化病1例

报告1例水源性肢端角化病。男,49岁,汉族。双手、手腕部皮肤接触水约1分半钟后出现米粒或芝麻大小苍白色及肤色丘疹,双手掌及手指侧缘起皱发白,增厚,时间越长皮损增多越明显,融合增大更明显;水温越高,皮损出现速度越快。无痒痛,轻度胀紧感,停止浸水并擦干后,皮疹逐渐消退,约15分钟后完全消退。高渗氯化钠溶液浸泡试验(-)。诊断:水源性肢端角化病。     

水源性肢端角化病二例

水源性肢端角化病是一种少见的角化性皮肤病,我们诊治2例,现报告如下。     

水源性肢端角化症

患者男,28岁。半年前开始双手接触水数分后钟局部出现丘疹和斑块,伴肿胀,脱离水源约15min后,皮损及不适感消失。临床表现为:双手掌可见细小、白色的扁平丘疹及斑块。皮损组织病理示:表皮角质层明显增厚,棘层肥厚。诊断:水源性肢端角化症。     

水源性肢端角化病一例

患者,女,63岁。手背部遇水后出现白色角化肿胀性丘疹20余年,干燥后皮损逐渐消退。水桶征阳性。诊断：水源性肢端角化病。     

肢端角化性类弹性纤维病

患者女性，４７岁。主诉：双手背、手腕屈侧、双足背丘疹２年。现病史：患者２年前开始在双手背、手腕屈侧、双足背内外侧出现半透明的丘疹，逐渐增大，无症状。半透明丘疹可逐渐转变为白色扁平丘疹。丘疹逐渐增多，反复有新起丘疹。患者为家庭劳动妇女。否认家族中有相同疾病者。体格检查：全身系统检查未见异常。皮肤科检查：双手背，大小鱼际处多发扁平白色扁平角化丘疹，呈“铺路石”样，部分皮疹融合（见照片１）。皮疹分布以背屈侧交界处为显著。双手腕屈侧半透明丘疹，为半球形粟粒至绿豆大小，表面光滑（见照片２）。双足缘内外侧于…     

水源性肢端角化症1例

水源性肢端角化症是一种由水诱发的极为罕见的皮肤病,在20世纪90年代首次披露[1],国内文献报道极少。笔者临床遇到1例,现报道如下。     

水源性肢端角化症1例

患者男,31岁,双手接触水数分钟后局部出现皮疹伴紧绷感,离开水约10min(皮肤干燥)后症状全部消退约半年。有慢性荨麻疹病史10年。皮肤科情况:双手皮肤未接触水前外观正常。用水和高渗氯化钠溶液浸泡进行"水桶征"试验:水浸泡(+);高渗氯化钠溶液浸泡(-)。取26℃水浸泡10min后诱发的右手掌尺侧缘皮损进行活检组织病理检查结果示:表皮角化过度,棘层轻度增厚,真皮汗腺分泌旺盛,部分汗管扩张。诊断:水源性肢端角化症。     

水源性肢端角化症

患儿,女,3岁.1年前双手出现暗红色质硬斑块,遇水后变白、肿胀、皱缩,干后恢复原状.无类似疾病家族史,外用0.025％维A酸乳膏,1个月后症状明显缓解.     

Acquired aquagenic syringeal acrokeratoderma: A case series of 10 patients

Aquagenic syringeal acrokeratoderma is a rare acquired disorder that predominantly affects young women. It is most commonly localised on the palms. It is characterised by translucent papules, oedematous plaques and keratoderma developing after brief exposure to water and resolving shortly after drying. We have observed 10 patients with this disorder within 13 months. We think that aquagenic syringeal acrokeratoderma is a more common condition than was originally anticipated as one can easily underdiagnose this entity due to the transient nature of its clinical findings.     

Aquagenic wrinkling of the palms in a cystic fibrosis carrier

A 16-year-old girl presented with a 3-year history of painful swelling and wrinkling of her palms on exposure to water. Physical examination, after less than 2 min of immersion, showed thickening and exaggerated wrinkling of the palms giving a whitish pebbly appearance. Aquagenic wrinkling of the palms was diagnosed. The patient was tested for the common cystic fibrosis mutations, and was found to be heterozygous for the arg117His cystic fibrosis mutation. Her sweat test was normal.     

Aquagenic keratoderma

Aquagenic keratoderma has been described as a transitory condition involving young females and defined clinically by the appearance of palmar lesions accentuated after immersion in water. According to previous case reports, these lesions are characterized histologically by hyperkeratosis and dilated eccrine ducts. Some aberration in the eccrine ducts has been suggested as a possible pathogenic mechanism. We report a new case in a female adolescent. With regard to the normal aspect of the epidermis in our case, the clinical changes should be ascribed to a structural alteration of the horny layer without any visible microscopy change or functional alteration of the stratum corneum involving the organism in the adolescence period.     

Transient reactive papulotranslucent acrokeratoderma associated with cystic fibrosis

A 20-year-old female with cystic fibrosis presented with a white marginal palmar eruption after exposure to water. There was no family history of keratoderma. A biopsy showed hyperkeratosis around dilated eccrine ostia. These features are similar to a recently described condition, transient reactive papulotranslucent acrokeratoderma. This is thought to be a variant of hereditary papulotranslucent acrokeratoderma, one of the punctate keratodermas. Association with cystic fibrosis has not been described previously.     

Case report: Treatment failure in a case of aquagenic syringeal acrokeratoderma

Aquagenic syringeal acrokeratoderma (ASA) is a rare, acquired, recurrent, and transient type of keratoderma that may occur after a few minutes of exposure to water. Herein, an 18-year-old male patient who had bilateral swelling and whitish plaques on his palms and soles is presented. The lesions on soles and heels developed within short time of immersion in water and resolved after 30 min with drying. In this case, all treatment methods, previously described in the literature in similar cases (i.e., aluminum salts, urea–salicylic acid including ointments, iontophoresis, and botulinum toxin) were ineffective. ASA is a condition that has an adverse effect on life quality. Alternative treatments are needed in ASA cases who are resistant to treatment modalities mentioned in the literature.     

Aquagenic wrinkling of the palms in cystic fibrosis and the cystic fibrosis carrier state: a case–control study

Background Aquagenic wrinkling of the palms (AWP) is hyperwrinkling occurring within 3 min of exposure to water. It is associated with cystic fibrosis (CF) and has been reported in a CF carrier. Objectives To ascertain if AWP is a sign of the CF carrier state and to test for an association between cystic fibrosis transmembrane regulator (CFTR) protein function and time to wrinkling. Methods Twenty‐one patients, 13 carriers and 15 controls were recruited. Hands were immersed in water and time to wrinkling was measured. An analysis of variance was performed with mean time to wrinkling as the dependent variable and CF status as the independent variable. Patients with a time to wrinkling of ≤ 3 min were defined as having AWP. A test of proportions was performed to assess if the proportion of patients with AWP varied between groups. Results Mean time to wrinkling was 11 min in controls, 7 min in carriers and 2 min in patients with CF. AWP was not seen in controls, but occurred in 80% of patients with CF and 25% of carriers. There was a significant difference between groups (P < 0·001). Conclusions The study demonstrated that AWP is a sign of both CF and the carrier state. It suggests that time to wrinkling decreases with decreased CFTR protein function. Patients presenting with AWP should be offered screening for both CF and the carrier state.     

Aquagenic acrokeratoderma

Background Aquagenic acrokeratoderma (AA) is a rare condition with female predilection that occurs after brief water exposure and disappears minutes to an hour after drying. The pathogenesis remains unclear. Methods Four Chinese patients with AA were reported and analyzed. Results There were 2 males and 2 females (age range: 14‐33 years) who presented with a 2‐week to more than 10‐year history of small white papules coalescing into edematous plaques on the hands; lesions appeared within 5‐10 minutes of water exposure, began to regress in 3–20 minutes and disappeared within 5 minutes to one hour after drying. Warm water provoked the lesions more rapidly than cold water. Lesions of a female patient could be triggered by detergent. In another female patient, lesions also involved the feet and were associated with palmoplantar erythema and hyperhidrosis. Biopsy from the lesion of one patient after water exposure revealed hyperkeratosis, mild hypergranulosis, and dilatated eccrine ducts. Biopsy from the lesion of another patient after drying showed normal epidermis and dermis. Two patients were treated with topical formalin 3% in alcohol, and two with 3% potassium aluminium sulfate solution with partial relief without any adverse effects. Conclusion AA may occur in both males and females and may involve the feet. Warm water triggers the lesions more rapidly than cold. Topical formalin 3% in alcohol and 3% potassium aluminium sulfate solution may be optional therapy.