进行性红斑角化症误诊1例

患儿男,７岁。双手足对称性潮红角化脱屑６年。患儿自１岁左右开始出现双手掌、足底片状潮红斑伴有脱屑,无明显瘙痒、疼痛,未予重视,后逐渐扩展至双手掌手背、足底足背,反复脱屑,冬季加重伴有皲裂,院外曾以“手足癣"及“掌跖银屑病”予以多种治疗,均未明显缓解。半年前出现指趾甲变形、断层,■窝出现同样红斑。患儿发育尚可,一般情况好,既往无其他疾病,家族中无类似病例。皮肤科情况:头发色黄较稀疏,口腔粘膜无皮疹,双手掌手背、足跖足背对称性大片潮红斑,边界清晰无浸润,手掌足跖可见皮肤角化肥厚,皮肤干燥,有脱屑,少量皲裂（图１,…     

进行性对称性红斑角化症1家系报道

患者女,26岁。出生后6个月发病,双手、足对称性红斑角化25年。体检:一般情况良好。双侧掌跖及腕关节屈侧对称性分布边界清楚的角化性红斑,边缘覆以少许脱屑,压之不褪色,指/趾甲均未受累。实验室检查未见异常。手背侧缘皮损组织病理示:表皮明显角化过度,伴轻度角化不全,颗粒层及棘层肥厚,表皮增生至同一水平线,真皮小血管轻度扩张充血,管周少量淋巴细胞浸润。诊断:进行性对称性红斑角化症。     

孪生姐妹同患进行性对称性红斑角化症

例1，女，2岁。生后3个月手足出现红斑，继而脱屑，后累及手、足背井渐加重。皮肤科情况：双倒掌、扶部、手、足背，四肢伸侧至肘、膝部有片状脱屑性红斑，呈对称性。掌防部潮红、浸润、肥厚伴角化、默裂，境界较清。例2，系倒1之挛生妹，生后6个月发病。皮肤科情况：双手、足背部及小腿伸例可见境界清楚的片状红斑角化，增厚脱屑性损害，掌伤部轻度积裂。孪生姐妹同患进行性对称性红斑角化症@孔玉沙$河南省周口地区医院@李娜$河南省周口地区医院     

掌跖角皮症伴发牙周病一例

患者男,13岁.因手足、肘膝关节伸侧红斑角化、脱屑13年来诊.患儿自生后8个月即发现双掌跖皮肤发红脱屑,缓慢加重,逐渐延至双手足背侧及腕踝关节皮肤,并有角化、皲裂.4岁时皮疹累及肘膝关节伸侧及臀部.其间牙龈红肿萎缩,乳牙逐渐脱落.6～7岁恒牙萌出,亦出现相同情况.曾多次求医,未确诊.既往无特殊病史,无特殊感染史.家族中无类似病史,父母为近亲结婚,其有兄弟2人,均健康.患儿为足月顺产,身高及体质量居正常下限.智力发育正常.各系统检查无特殊.肝肾功能、血脂等检查正常.皮肤科检查:双掌跖及其背侧、双腕踝关节皮肤红斑角化,尤以掌指及指指关节背侧明显.足底皮肤明显角化增厚,大片脱屑,有皲裂.肘膝关节伸侧红斑脱屑,皮损从双小腿外侧上延与膝伸侧皮疹相连,境界清楚,臀部亦有类似损害,呈地图样外观.指趾甲表面不光滑,有甲纵嵴,部分呈黑褐色,远端甲板可分离自行脱落.面部多数雀斑样损害.牙齿较大,排列不齐,松动,部分脱落.牙龈萎缩.口腔曲面断层X线全景片示:牙槽骨明显吸收.皮损病理检查示:表皮角化过度,不规则棘层增厚伴乳头瘤样增生,真皮浅层少量淋巴细胞浸润.     

弥漫性非表皮松解性掌跖角化病1例

1 临床资料 患者男,26岁.双手、足角化过度20余年.24年前无明显诱因出现双手十指发白,皮肤粗糙增厚,逐渐蔓延至指腹、手掌、手背、腕部、双足底、足背及踝部;约20余年前手、足皮肤增厚明显,呈蜡黄色斑块状,反复脱屑、皲裂,伴有疼痛,偶有瘙痒,双手活动受限,以右手为著;18年前出现指(趾)甲增厚、浑浊,口唇黏膜鲜红,口周出现红斑,伴少量脱屑;6年前左肘关节出现角化性红斑,上覆薄层片状鳞屑;3年前双手活动显著受限;1年前鼻背出现红斑,伴轻微瘙痒.     

残毁性掌跖角化病一例

患者20岁，女性，从2岁起掌跖皮肤出现红斑、增厚伴脱屑并逐渐加重，12岁时左手示指甲脱落并发生指背溃疡，溃疡无疼痛和压痛。目前双手拇指节末端杵样膨大。食指缩短，中指伸侧有蜂窝状凹陷，各手指关节伸侧对称性分布暗红色角化性斑疹；手掌合拢困难；足趾角化增厚边缘附黄色鳞屑，足跖部见胼胝样角化增厚斑块；皮损无瘙痒感。结合X线、实验室和组织病理检查，诊断为残毁性掌跖角化病。给予阿维A治疗，疗效较好，并对患者进行了长期随访。     

进行性对称性红斑角皮症伴癫癎1例

2002年5月我科诊治1例进行性对称性红斑角皮症患儿,伴发癫癎,有明显智力障碍,现报告如下. 患儿女,5岁.手足出现红斑、角化、脱屑4年余,伴有抽搐及智力障碍.患儿自出生后即发现掌跖红斑、角化、脱屑,并逐渐扩大,延及手足背,伴多汗.     

进行性对称性红斑角皮症1例

1 临床资料 患儿男,14岁.双掌跖、膝、肛周红斑角化近十年.从4岁起患者无明显诱因双手掌、足跖弥漫性红斑及角化过度,伴有潮湿、多汗,随年龄增长逐渐加重,并扩大累及双手足指趾背、足背.7岁时皮损开始累及双膝关节伸侧及肛周、腰部皮肤.     

弥漫性非表皮松解性掌跖角化病1例

1病历摘要 患者女，42岁。闲双侧掌跖角化40余年，于2006年9月来我院就诊。患者自婴儿期起无明显诱凶双手掌出现对称性片状红斑，角化，形状不规则．受累皮肤粗糙增厚，同时累及双足和甲板，指（趾）甲增厚、浑浊，呈灰黄色。皮损逐渐向周同扩展，至青春期时已经累及双手整个掌侧、腕部及于背的大部分，双足跖和足背亦呈弥漫性角化过度，跖部较重，皮损呈对称性，     

先天性厚甲综合征1例

先天性厚甲综合征１例袁阳，陈晶大连市皮肤病防治所（邮政编码１１６０２１）患者女，１９岁。指（趾）甲肥厚１９年。患者出生后，双手足指趾甲即增厚，伴疼痛。５年后，双掌趾出现角化，腹部、四肢伸侧发生毛囊性丘疹，无明显季节性。５年前，双腋下、腹股沟、足缘发生...     

Case of remitting seronegative symmetrical synovitis with pitting edema (RS3PE syndrome) showing dermatomyositis-like eruption

A 73-year-old woman developed linear erythema at the sites of scratching-induced scars on the bilateral thighs 2 weeks before the initial consultation. Subsequently, edematous erythema developed in the upper eyelids, dorsum of the nose and the face, and pitting edema in the dorsum of the bilateral hands and feet. The C-reactive protein (CRP) level was 8.2 mg/dL and erythrocyte sedimentation rate (ESR) 121 mm/h. The antinuclear antibody titer was 1:160, and rheumatoid factor (RF) and anti-Jo-1 antibody were negative. X-ray examination of the bilateral hands showed neither narrowing of the joint spaces nor bone erosion. Ga scintigraphy showed synovitis of the bilateral wrists. A diagnosis of remitting seronegative symmetrical synovitis with pitting edema syndrome (RS3PE) was made. The erythema disappeared after diclofenac sodium administration. However, because the joint swelling and pitting edema did not improve, p.o. administration of prednisolone (20 mg/day) was initiated. The CRP and ESR levels normalized 2 months after the initiation of administration, and pitting edema disappeared after 3 months. We report this case because linear erythema like that observed in dermatomyositis has not been described as eruptions associated with RS3PE.     

Erythema scarlatiniforme desquamativum generalisatum

Erythema scarlatiniforme desquamativum generalisatum (Féréol-Besnier disease) is a rare skin disease characterized by generalized erythematous rash with subsequent desquamation. An 86-year-old woman presented with generalized erythema followed by an extensive, scarlatiniform peeling especially of the hands and feet. This generalized episode may be followed by erythema scarlatiniforme desquamativum localisatum recidivans, which is a recurring variant of the disease, localized to the hands and feet.     

Toxic erythema of palms and soles associated with high-dose mercaptopurine chemotherapy

We encountered a case of distinctive palmar-plantar erythema with desquamation of the fingers in a patient receiving high-dose mercaptopurine combined with allopurinol. He was receiving 400 mg/d of mercaptopurine with 200 mg/d of allopurinol when a painful, livid erythema involving his hands and feet developed. Over the ensuing 24 hours, desquamation of the distal fingertips was noted. The mercaptopurine was discontinued and the patient was treated with topical fluocinonide ointment under occlusion. Over the next 96 hours, the erythema and pain resolved entirely. To date, this is the eighth case of a painful desquamating erythema of the palms and soles occurring as a complication of chemotherapy. We suggest that high-dose mercaptopurine combined with allopurinol that blocks xanthine oxidase, a necessary enzyme in the catabolism of mercaptopurine, was responsible for our patient's clinical presentation.     

Palmoplantar keratoderma of Sybert

A 13-year-old boy and a 7-year-old boy, who are brothers, presented with a life-long history of erythema, hyperkeratosis, and desquamation of the hands and feet. Symptoms improved with the use of topical glucocorticoids and keratolytics. PPK of Sybert is characterized by palmoplantar hyperkeratosis with transgrediens, autosomal dominant inheritance, and the absence of associated systemic features.     

Dorsal sensory impairment in hands and feet of people affected by Hansen's disease in Israel

INTRODUCTION: Sensory testing in people affected by Hansen's disease is usually performed on palms and soles only. In Israel, both palmar/plantar and dorsal aspects of limbs are routinely tested. OBJECTIVES: The aim of this study was to describe the magnitude of dorsal sensory impairment (SI) in limbs and compare the frequency of SI on palms and soles with that on the dorsum of hands and feet. DESIGN: In a cross-sectional study, limbs of 140 patients registered at The Israel Hansen's Disease Centre during the years 1999-2003 were tested for their sensory status. Both palmar/plantar and dorsal aspects were tested using Semmes-Weinstein monofilaments. SI was defined as not feeling stimuli applied with the 2 g monofilament. RESULTS: SI was detected on the dorsum in 43% of sites on hands and only in 27% on palms. 64% of sites on dorsum of feet had SI compared to 53% on the soles. SI was detected in up to 18% in hands with no palmar SI, and in 6% of feet with no plantar SI. Furthermore, SI on palms and soles was found to be accompanied by dorsal SI in all hands and in 97% of feet. CONCLUSION: SI on dorsum of limbs occurs more frequently than SI on palms and soles. Therefore sensory testing should also consider inclusion of the dorsal aspect of hands and feet.     

缓和性血清阴性对称性滑膜炎伴凹陷性水肿综合征一例并文献复习

目的了解缓和性血清阴性对称性滑膜炎伴凹陷性水肿综合征的临床表现。方法分析1例缓和性血清阴性对称性滑膜炎伴凹陷性水肿综合征患者临床表现、实验室检查及治疗反应。结果患者男,54岁,表现为对称性多关节炎及肢端水肿。血清类风湿因子、抗核抗体、抗ds—DNA抗体、抗合成循环瓜氨酸多肽抗体、免疫球蛋白、补体均正常。x线检查示双手掌指骨骨质破坏。糖皮质激素和免疫抑制剂治疗效果好。结论缓和性血清阴性对称性滑膜炎伴凹陷性水肿综合征是一组异质性疾病。     

Is acquired symmetrical acrokeratoderma a new dermatosis? Two case reports and Chinese literature review

Background A series of cases characterized by symmetrical acral hyperkeratosis, mainly involving the dorsal surface of the hands, feet, and wrists, but sparing the palmoplantar areas, as well as rapid immersion upon exposure to water have been recently described in China, but similar disorders have not been reported in the English literature. Methods The clinicopathologic features of two cases of acquired symmetrical acrokeratoderma were reported and 27 Chinese patients were reviewed. Results The disorder typically occurred in young or middle aged men. Brown to black hyperkeratotic patches were symmetrically distributed particularly on the wrists and dorsum of hands, fingers, and feet, but without involvement of palms and soles. The lesions became whitish with mild swelling immediately after contact with water and improved generally in winter. Histopathologic examination revealed epidermal hyperkeratosis, acanthosis, and papillomatous hyperplasia, as well as superficial perivascular lymphohistiocytic infiltrate. Main ultrastructural features of the immersed lesion were epidermal hyperkeratosis and spongiosis with partial split of the desmosomes. Conclusion This disorder may be a new dermatosis, and the term “acquired symmetrical acrokeratoderma” could exactly reflect its clinicopathologic characteristics.     

Progressive symmetrical erythrokeratodermia - Case report

Progressive symmetrical erythrokeratodermia is a rare autosomal dominant genodermatosis with variable penetrance described by Darier in 1911. It is characterized by erythematous and keratotic plaques, sharply defined and symmetrically distributed along the extremities, buttocks and, more rarely, on the face. We report a case of a 55-year-old patient with lesions on the dorsum of the hands, interphalangeal pads, wrists, groin and back feet. This case demonstrates a rare and late diagnosis, clinical profusion and presence of familiar involvement.     

多发性硬下疳合并二期梅毒1例

患者男,34岁。阴茎、阴囊反复起溃疡6个月,躯干、大腿起红斑5d。查体:阴茎及阴囊可见多处浅溃疡,直径0.5～1.0cm,界清,边缘整齐,呈软骨样硬度,无触痛。躯干、大腿见广泛分布类圆形淡红斑,大小不一;双手足未见红斑和脱屑。实验室检查:TPPA(+),RPR1:128(+),HIV抗体阴性。HSV-IIgG(+),HSV-IIIgG(+),HSV-IIgM(-),HSV-IIIgM(-)。诊断:多发性硬下疳;二期梅毒。予苄星青霉素240万U分两侧臀部肌注,1次/周,连用3次后皮损消退。     

Keratoderma hereditaria mutilans (vohwinkel's syndrome)

We report two cases of Vohwinkel's syndrome in son and mother presenting with a progressive palmoplantar keratoderma with 'honeycomb' appearance associated with constricting bands encircling digits of hands and feet along with keratotic plaques on dorsum of hands and feet. Both had associated sensorineural deafness.