后足截骨结合泰勒空间外固定支架矫正严重马蹄足畸形临床研究

目的探讨后足截骨结合数字外固定支架的数字化矫正在严重马蹄足畸形中的应用效果。方法于2013年5月—2019年5月,采用后足截骨加泰勒空间外固定支架(Taylor Spatid Frame,TSF)治疗的19例严重马蹄足患者(23足),其中主要表现为马蹄畸形>40°13足;马蹄合并前、中足明显内收及内翻>20°10足。以马蹄畸形为主合并轻度内翻及旋转的患足实施后足U形截骨加标准足踝TSF构型及安装,参数测量时以距下关节前方为畸形旋转中心及起始点,以全足与胫骨对照进行矫正。马蹄合并前足内收、旋转及后足内翻,实施V形截骨。足部空间足够时,在足部及跨踝关节安装6+6串联TSF,分别以距舟关节内侧缘及距下关节前方为畸形旋转中心及起始点,同步矫正前足及马蹄畸形;足部空间狭小时,先以前足与胫骨为对照安装TSF矫正前足畸形。然后再次变换固定模式,将足与胫骨对照矫正足的马蹄及内翻畸形。结果所有病例均完成治疗,畸形矫正85%~100%,平均93.3%,无严重的并发症。获得0.5~1年随访20足,依据课题组马蹄足畸形矫正标准:优7足、良11足、可2足、差0足;依据ICFSG评分系统:优3足、良14足、可3足、差0足。结论后足截骨安装外固定支架牵伸矫正严重的马蹄足畸形能够获得较好的疗效,并发症少。应用数字外固定及数字化矫形技术,可以更加精准地矫正严重的马蹄足畸形,容易被医生掌握,是足踝外科的一个发展方向。     

Syndrome of giant deratoma of the feet (syndroma cornuum giganteum dedum)

The syndrome of giant keratoma of the feet with disseminated keratotic papules on the thighs and legs, lack of fusion of the posterior arch of the L5, S1 vertebrae, varus deformity of feet and (congenital?) deformity of metatarsal bones and skeletal atrophy of feet, supernumerary 6th fingers and toes, extreme gothic palate, scanned speech, and low oscillometric values on the legs was described in a 28-year-old man. Good results were achieved after electrocoagulative removal of the keratomas and systemic treatment with Tigason and high doses of vitamin E.     

Orofacial granulomatosis and erythema multiforme in an adolescent with Crohn's disease

The cutaneous manifestations of Crohn's disease are myriad. A 15‐year‐old girl presented with recurrent lip swelling and eventual development of diarrhea and targetoid macules on the palms, feet, and back. She was finally diagnosed with Crohn's disease in the setting of a clinical presentation and histopathology consistent with orofacial granulomatosis and erythema multiforme. We review the literature and summarize reported occurrences of these cutaneous diseases in children with Crohn's disease.     

Hereditary palmoplantar keratoderma (four cases in three generations)

A 39-year-old man reported with progressive thickening of the skin of the hands and feet and an inability to flex his hand. It was largely asymptomatic; however, brisk walking caused excessive sweating, pain, and widening of the fissures on the soles of the feet. He was unable to walk barefooted. According to his mother, the first episode presented with blistering at 7 days of age. Ever since, the condition has steadily worsened to acquire the current status. He was married at the age of 18 years, and had a stillborn child 18 months afterwards. Presently, he has three children, two girls aged 14 and 12 years and a son aged 10 years. Both the daughters are similarly affected. While cataloguing the details of the pattern of inheritance, the mother of the index case was also found to be affected (Fig. 1). The natural history of the disease was identical. Examination of the palms was marked by pronounced thickening of the skin resulting in the masking of palmar creases. The thickening was well demarcated and its margins were prominent and surrounded by an erythematous halo. The color of the skin was yellow and waxy (Fig. 2a). Contractures were present on all the fingers; nevertheless, the deformity of the middle and distal interphalangeal joints of the little finger was prominent. The soles of the feet had a similar morphology. In addition, marked fissuring was obvious (Fig. 2b). His daughters had an identical affliction of the palms and soles. The texture and morphology of the nails were normal. Light microscopy performed on scrapings from the fissures, mounted on 10% potassium hydroxide, revealed mycelia (hyphae) and spores. Hematoxylin and eosin-stained microsections from the palms and soles showed exquisite changes in the epidermis characterized by considerable uniform orthohyperkeratosis. Hypergranulosis and acanthosis were other associated changes. In addition, perinuclear vacuolization and keratohyalin granules of varying sizes and shapes were located at the periphery of the cells. A sparse mononuclear infiltrate was located at the dermo-epidermal junction. Hyphae and spores of fungi were also identified in the stratum corneum (Fig. 3). Itraconazole, 400 mg/day in two equally divided doses, was administered with major meals for 7 days. In addition, high doses of vitamin A (100,000 IU) were given daily for 2 weeks, supplemented by 12% salicylic acid (Salicylix SF12) ointment for daytime application and an ointment containing 6% coal tar and 3% salicylic acid (Salytar) for night-time application. This treatment is useful in recalcitrant cases.     

REPORT OF HEAT MOULDED FOOTWEAR FOR A PATIENT WITH EPIDERMOLYSIS BULLOSA

A patient with epidermolysis bullosa, which caused gross deformity of the feet and posture, was fitted with heat moulded footwear to enable her to regain independent movement and, as far as possible, to correct postural abnormalities.     

Acanthosis Nigricans Associated with Hepatolenticular Degeneration

Acanthosis nigricans and hepatolenticular degeneration (Wilson's disease) developed simultaneously in a 16-year-old boy. The diagnosis of Wilson's disease was based on the clinical presentation, including Kayser-Fleischer ring, hypoceruloplasminemia, hypocupremia, and hypercupriuria. His skin lesions were characterized by thick, dark brown, verrucous plaques on the dorsa of both feet, the neck, axillae, and groin. The histological findings were compatible with acanthosis nigricans. Six months after treatment with D-penicillamine, two grams per day, his skin lesions and neurological symptoms were much improved and no complications were observed.     

Radiological changes in hands and feet in disabled leprosy patients: a clinico-radiological correlation.

Seventy-six consecutive leprosy patients with disabilities were subjected to radiological examination of hands and feet, and bone changes were found in 63 of them (82.9%). Specific, nonspecific and osteoporotic bone changes were observed in 22.4%, 78.9% and 28.9% of cases respectively. Bone cysts (10.5%), subarticular erosions (10.5%) and enlargement of nutrient foramina (5.3%) were the common specific bone changes whereas bone absorptive changes (59.2%), soft tissue changes (39.5%) and concentric absorption (39.5%) were the most frequent nonspecific bone changes. Specific bone changes were more common in older patients (age 40 years) and nonspecific bone changes correlated with duration of disease, duration of deformity, and disability index. Osteoporotic bone changes were found to be affected by ageing and severity of disability of hands and feet.     

Multiarticular Tophaceous Gout with Severe Joint Destruction: A Pictorial Overview with a Twist

Tophi are the visible dermatological signs of gout. A case of tophaceous gout in a middle-aged man with no other metabolic derangement is being presented with multiple tophi on the hands and feet overlying joints as well as on the fingers and toes. We thought it to be of educational value to demonstrate needle-like crystals of urate by polarizing microscopy. X-rays of hands and feet showed dramatic destructive changes. The patient presented with mottled hypopigmentation on anterior and posterior knees and dorsa of hands and feet where he applied hot “aankda” leaves and covered them with bandage resulting in irritant dermatitis with postinflammatory hypopigmentation. This proved to be a red herring in this case.     

Radiological changes in psoriatic arthropathy

Forty-one cases of psoriatic arthropathy (PA) were selected for the study. Biopsy, x rays of hands, feet, cervical and dorsolumbar spine, sacro-iliac joints and routine investigations were carried out. Clinical diagnosis of psoriasis was confirmed histopathologically. Radiological changes in order of frequency were most common in feet - 26/41, hands - 24/41, sacro-iliac joints -11/41, dorso-lumbar spine - 4/41 and cervical spine - 3/41. Distinctive radiological changes were seen in psoriatic arthritis.     

A Case of Reticulate Acropigmentation of Kitamura: Dowling Degos Disease Overlap with Unusual Clinical Manifestations

Reticulate hyperpigmentary disorders are a group of rare genetic pigmentary abnormalities which includes reticulate acropigmentation of Kitamura (RAPK), Dowling-Degos disease (DD), reticulate acropigmentation of Dohi (RAPD), Haber''s syndrome, and Galli-Galli disease. A 25-year-old male presented with asymptomatic dark-colored lesions on his hands and feet with light-colored skin lesions involving the trunk since three years. Dermatological examination revealed hyperpigmented macules in a reticulate pattern involving the dorsa of the hands and feet, front and sides of the neck, axillae, periorbital region, and groin. Multiple pits were present over both palms, with breaks in dermatoglyphics. He also had multiple nonacne facial scars predominantly on the nose and malar areas. The patient had overlapping features of RAPK and DDD. In addition, he also had hypopigmented macules and acneiform facial scars. Such an overlap of features of reticulate pigmentation has not been previously reported in the literature.     

Cryopyrin蛋白相关周期性综合征一例

患者女,29岁.反复面部、躯干弥漫性红斑26年.手、足关节变形14年.皮疹每日发作,伴寒战、低热、眼红、关节痛.体检:身材矮小,面容特殊,呈塌鼻,额部凸出样,双眼结膜充血,而部、躯干弥漫性红斑,手、足关节,变形.面部经冷风刺激后,皮疹加重呈典型风团.根据典型症状诊断为Cryopyrin蛋白相关周期性综合征.     

系统性硬皮病肢端型与弥漫型73例临床特点分析

目的探讨系统性硬皮病(SSc)肢端型与弥漫型的临床特点及差异。方法回顾性分析47例肢端型和26例弥漫型SSc患者的临床资料,并比较两型差异。结果肢端型∶弥漫型≈1.81∶1;指趾骨吸收/溃疡、关节畸形/固定、趾雷诺现象、ACA(+)等发生率肢端型高于弥漫型(P<0.05~0.01);关节炎、肌炎、间质性肺炎、二/三尖瓣返流、肾脏受损等弥漫型高于肢端型(P<0.05~0.01);2例分别死于心衰和尿毒症,均属弥漫型。结论两型除皮肤变硬部位不同外,肢端型SSc以指、趾骨吸收、溃疡、关节畸形、固定功能障碍为显著临床特点;弥漫型SSc则以关节炎、肌炎、肺、心脏受累为显著临床特点。心肾受损可能是SSc致死的主要因素。     

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。     

银屑病关节炎临床及放射学分析

本文对40例银屑病关节炎进行临床及放射学分析,银屑病皮损先于关节症状者29例,在其后者5例,与之同时发病者6例.主要侵犯指、趾及手足诸关节.27例有不同程度甲病变.放射学特征,系手、足末端指(趾)间关节受累,为关节间隙狭窄、骨性侵蚀及骨增生.类风湿因子试验阴性占94.7%.并讨论了银屑病关节炎与类风湿性关节炎的鉴别诊断.     

Malignant Melanoma Developing in an Area of Palmoplantar Keratoderma (Greither's Disease)

We report a case of malignant melanoma arising on the hyperkeratotic sole of a patient with palmoplantar keratoderma (PPK). Hyperkeratotic lesions were also seen on the dorsa of both hands and feet and the extensor aspects of elbows and knees. The patient's PPK appeared to have been transmitted by an autosomal dominant gene. Histologically, the hyperkeratotic lesions showed acanthosis, marked hyperkeratosis without parakeratosis, and hypergranulosis. All the findings corresponded to those of Greither's disease with malignant melanoma.     

Clonal identity between skin and synovial tissue in a case of mycosis fungoides with polyarthritis

Polyarthritis in the presence of a cutaneous T-cell lymphoma is a rare phenomenon. We describe a case of mycosis fungoides with development of a symmetric erosive polyarthritis of the small hand joints and feet, diagnosed as rheumatoid arthritis. An identical monoclonal T-cell population in the skin and in the synovium was detected by T-cell receptor gene rearrangement analysis, illustrating articular dissemination of lymphoma cells. Differentiating mycosis fungoides-associated arthritis from rheumatoid arthritis may have important implications for treatment. Based on this case, the relevant literature, and the newest disease concepts, pathogenic mechanisms and therapeutic options of mycosis fungoides-associated arthritis are discussed.     

去除部分甲母质治疗钳形甲35例疗效观察

目的观察部分切除甲母质治疗钳形甲及其所致甲沟炎的临床疗效。方法无炎症的钳形甲患者予部分拔甲和部分切除甲根部的甲母质(即钳形甲矫正术);钳形引起甲沟炎的化脓期或慢性肉芽肿期患者行拔除部分趾甲和清创引流术,待炎症控制且创面愈合1个月后行钳形甲矫正术。结果共治疗22例计35病足,随访12个月,钳形甲畸形及疼痛症状均消失,未见复发。结论钳形甲矫正术简单易操作,患者恢复快,无复发,值得临床推广应用。     

硬皮病样表现伴关节挛缩的慢性移植物抗宿主病一例

患儿,男,5岁。双手足质硬、红斑、关节挛缩2年。患儿自幼反复罹患肺脓肿并伴有发热,4年前行基因检测,诊断为JAK3基因相关重型联合免疫缺陷症,遂行异基因造血干细胞移植。2年前先于足部出现脱皮,后双手足背、腕、肘、膝及髋关节处出现红斑、脱皮、硬化,伴手足关节挛缩。皮肤组织病理示移植物抗宿主反应并病毒感染。     

Acroangiodermatitis (pseudo-Kaposi's sarcoma) in an HIV sero-positive patient with syphilis and hepatitis C virus coinfection: clinical and dermatopathological features

Acroangiodermatitis is an angioproliferative disease usually related to chronic venous insufficiency, and it is considered a clinical and histological simulator of Kaposi''s sarcoma (KS). Immunohistochemistry is the suitable method to differentiate between these two entities. It reveals the following immunostaining profile: immunopositivity with anti-CD34 antibody is restricted to the vascular endothelium in acroangiodermatitis, and diffuse in the KS (endothelial cells and perivascular spindle cells); immunopositivity with anti-HHV-8 only in KS cases. We report the case of an HIV seropositive patient without apparent vascular disease, who presented violaceous and brownish erythematous lesions on the feet, and whose histopathology and immunohistochemistry indicated the diagnosis of acroangiodermatitis.     

Celalettin Muhtar Ozden (1865–1947): his life,works and contributions to the study of dermatophytes

Background Most of the distinctive studies concerning dermatophyte infections were carried out at the end of the 19^th century. One of the contributors of these studies was the Turkish dermatologist Dr. Celalettin Muhtar Ozden. The aim of this study was to introduce his life, works, and contributions to dermatology. Methods Dr. Ozden's original works and published memories about him were evaluated. Results After graduating from the Military Medical School he was sent to Paris to carry out his studies on skin and syphilitic diseases in 1889 by the School. During his practice in Dr. A. Fournier's dermatological clinic at St. Louis Hospital (1889–1892), he diagnosed cases of trichophytosis that affects hands and feet. He published his findings collectively, in which, he presented information regarding the distinctive diagnosis and treatment of the disease, through a series of 25 cases, 18 of which were observed by himself in 1892. After returning to his homeland, Dr. Ozden trained many medical students through the last years of the Ottoman Empire to the first years of the Republic of Turkey. Conclusion His publication dated 1892 is characterized by being the first detailed work on dermatophyte infections affecting hands and feet. Today, he is well known by dermatologists in Turkey and his name has been immortalized through the term, the ‘Celal Muhtar's disease.’ It is important that this famous Turkish physician should keep his rightful place in the medical history of the world as well.