Treatment of scleromyxoedema with hydroxychloroquine

Background: Scleromyxoedema is a rare disease of unknown aetiology that is characterized by deposition of mucin and sclerotic induration of the skin; it is associated with paraproteinaemia. Patients suffer from progressive disability due to immobilization and cosmetic disfigurement. Treatment of scleromyxoedema is a therapeutic challenge. The antimalarial hydroxychloroquine has a rapid and reliable effect in reticular erythematous mucinosis. Patients and methods: Four consecutive patients (two women, two men; median age: 50 years) with scleromyxoedema, three of them with IgG λ paraprotein, were treated with hydroxychloroquine. Treatment was initiated with 600 mg p. o. for 10 days, followed by 400 mg for at least 4 weeks, and 200 mg thereafter. Results: Complete remission of skin manifestations was achieved in one patient, whereas three patients achieved a partial remission of 61+, 5 and 25 months' duration. Notably, three patients felt increased mobility and reduced firmness of skin during the first week of treatment, which was reflected in a rapid reduction in dermal thickness. In one patient, dysphagia was reverted as evidenced by normalization of oesophageal clearance. Paraproteinaemia was not influenced at all. Side effects included one case of electroretinogram abnormalities after 19 months of therapy and one case of leucopenia after 3 months. Conclusion: Hydroxychloroquine is an effective form of therapy for scleromyxoedema, leading to rapid and prolonged alleviation of symptoms.     

The effect of subhypnotic doses of propofol on the incidence of pruritus after intrathecal morphine for Caesarean section

The effect of subhypnotic doses of propofol on intrathecal morphine-induced pruritus was studied in a prospective, randomly allocated, double-blind controlled trial. Fifty-eight women undergoing elective lower segment Caesarean section for a singleton fetus received spinal anaesthesia with 2.5 ml hyperbaric 0.5% bupivacaine and 0.2 mg of preservative-free morphine. They then received propofol 1 ml (10 mg) or Intralipid 1 ml (control group) intravenously after delivery. Pruritus was assessed using a five-point verbal rating scale at hourly intervals for 8 h. A second dose of their allocated treatment drug was administered at the first recording of significant pruritus. The pruritus score was reassessed after 5 min and the treatment was repeated if pruritus remained. There were no differences between the groups in the onset of pruritus or its successful treatment. No adverse side-effects were associated with this dose of propofol. There were no differences in the incidence of post-operative nausea and vomiting between the two groups. Subhypnotic propofol is not an effective treatment for intrathecal morphine-induced pruritus in women following Caesarean section.     

Leishmaniasis of the lip in a patient with Down''s syndrome

Cutaneous leishmaniasis is an endemic protozoan infection in Sardinia, one of the major islands of the Mediterranean Basin. The main causative strain in this country is Leishmania infantum, which rarely involves mucocutaneous areas, but has the potential to cause visceral leishmaniasis. An atypical leishmaniasis involving the inferior lip of a 57-year-old female with Down's syndrome was observed at the Dermatology Department of Cagliari (italy). The diagnosis was mainly based upon histopathological examination, revealing intra- and extra-cellular leishmania amastigotes. The leishmania infantum zymodeme MON-111 was identified by isoenzymatic characterization. Laboratory investigations revealed a normal complete blood count and biochemistry profile, except for an inverted CD4/CD8 ratio. Treatment with meglumine antimoniate 60 mg/kg/day (Glucantime) intramuscularly for 15 days, followed by intralesional administration 1 ml weekly for 4 weeks led to complete recovery. No relapses were observed at 6-month follow-up. The unusual localization is likely to be a reflection of the uncommon site of inoculation of the protozoa, transmitted by bites from flying vectors. Nevertheless, the presence of Down's syndrome in our patient may have contributed to the atypical presentation by traumatic exacerbation of the lesion, due to repeated auto-induced microtraumas of the inferior lip accompanied by subclinical immunodeficiency. In fact, the specific immune response to Leishmania infection depends on a host-cell-mediated immune response, reported as defective in Down's syndrome patients. Differential diagnosis and early detection of the infection are necessary in order to start effective treatment and prevent more serious complications.     

腓动脉皮支逆行岛状皮瓣的解剖与应用改进

目的探讨腓动脉皮支逆行岛状皮瓣的解剖学基础及临床应用效果。方法采用新鲜及防腐尸体下肢共25肢,对腓动脉皮支进行解剖学观测。于2003年9月~2005年6月收治足跖趾关节处软组织缺损患者10例,男8例,女2例。年龄21~55岁。其中背侧6例,跖侧4例,缺损范围10cm×6cm~15cm×10cm。应用带有外踝上11.0±1.7cm腓动脉固定皮支的腓动脉皮支逆行岛状皮瓣修复,皮瓣范围11.0cm×6.5cm~16.0cm×11.0cm。供区6例直接减张缝合,4例行自体游离皮片覆盖。结果解剖学观测外踝上11.0±1.7cm自腓动脉发出一固定皮支,起始点直径1.45±0.12mm,入深筋膜点距腓肠神经营养血管皮瓣轴线15.70±1.20mm。10例患者术后皮瓣均成活,血运良好,无静脉回流障碍;供区愈合良好。术后获随访6~12个月,皮瓣外形良好,质地、色泽与周边相似,足功能恢复良好,两点辨别觉11~18mm。结论腓动脉皮支逆行岛状皮瓣经改进后血运好,逆转距离长,修复范围大,操作简便,为临床修复足中远端软组织缺损提供了一种新方法。     

An Unusual Form of Skin Tuberculosis Following B.C.G. Vaccination

A 25-year-old female has had brown to erythematous telangiectatic patches and grouped papules on her face, neck, arm, and trunk since childhood following B.C.G. vaccination. Histopathologically, the lesions consisted of hyperkeratosis, slight acanthosis, tuberculoid granulomas with some Langerhans type giant cells in the mid-dermis. Although various forms of cutaneous tuberculosis after B.C.G. vaccination have been reported, it was difficult for us to assign the patient's skin lesion to any specific disease entity. Remission of her cutaneous lesions occurred clinically and histopathologically after treatment with isoniazid and rifampin.     

带蒂组织瓣转移手术修复肢体软组织缺损35例分析

目的　分析带蒂组织瓣转移手术修复肢体软组织缺损创面的疗效。方法　选择肌瓣、岛状皮瓣、岛状肌皮瓣转移 ,直接修复缺损或转移肌瓣表面植皮覆盖。结果　 2 9例慢性骨髓炎和 6例外伤后软组织缺损创面 ,肌瓣、岛状皮瓣、岛状肌皮瓣转移修复疗效满意。结论　带有营养血管的肌瓣、皮瓣肌皮瓣转移手术 ,一期修复肢体创伤、骨髓炎等所致的骨缺损腔及软组织缺损创面 ,效果好 ,因不需吻合血管 ,手术相对简单 ,临床使用安全。     

A leg ulcer as manifestation of metastatic squamous cell vaginal carcinoma

Cutaneous metastasis of vaginal carcinoma is extremely rare. So far, the total number of reported skin metastasis of vaginal carcinoma is only one. We present another case with an unusual manifestation of vagina carcinoma metastasis: skin metastasis presenting as a leg ulcer on the lower leg.     

Ano-cutaneous flap repair for complex and recurrent supra-sphincteric anal fistula.

BACKGROUND: Suprasphincteric fistulae remain the most difficult to cure. OBJECTIVES: The purpose of this study was to evaluate the healing rate of suprasphincteric anal fistula treated by ano-cutaneous advancement flap repair, and the impact of this procedure on continence and quality of life. METHOD: Sixteen patients with complex, recurrent or chronic suprasphincteric fistulae associated with significant tissue damage (necrotizing fasciitis, keyhole deformity and anal stenosis) or who had failed previous surgical procedures were treated by ano-cutaneous flap closure. They were assessed pre and postoperatively by the treating surgeon for wound healing and fistula recurrence and later followed up by phone interview using the St Mark's Hospital incontinence score and the Perianal Disease Activity Index (PDAI) as indicators of treatment outcome. RESULTS: Fifteen patients had successful healing of their fistula with the cutaneous flap, with recurrence in only one. The most common short-term complications were minor graft site wound separation, which healed in all cases without intervention, and wound pain, which settled over time and was not associated with recurrence. Continence improved for almost 70% of the patients, with a significant reduction in St Mark's incontinence scores (t = 2.62, 15 d.f., P = 0.02). PDAI also decreased significantly (t = 7.55, 15 d.f., P < 0.001), demonstrating improvement in quality of life for most patients. CONCLUSION: Ano-cutaneous flap can achieve healing of complex and recurrent suprasphincteric anal fistula in patients who had previously failed at other forms of treatment thus improving their quality of life and continence.     

Periocular involvement in cutaneous leishmaniasis

OBJECTIVE: Although cutaneous leishmaniasis (CL) occurs mostly in the facial area, periocular involvement accounts for 2-5% of the facial lesions. CL lesions localized in the periocular region can easily be confused with various other diseases. The aim of this study was to examine the frequency of periocular involvement in CL in the Cukurova region of Turkey, as well as the clinical characteristics, diagnosis and methods of treatment of this disease. METHODS: Between December 1998 and December 2004, patients who were diagnosed with CL were evaluated prospectively with respect to periocular involvement. RESULTS: From the 2066 patients evaluated with CL, 2622 lesions were identified. In 59 (2.9%) of these patients, a total of 66 (2.5%) lesions were located in the periocular area. Thirty-two (48.5%) of these lesions were of the papular type, 15 (22.7%) the nodulo-ulcerative type, 10 (15.2%) the plaque type, and nine (13.6%) the nodular type. Dacryocystitis was identified in four patients with periocular involvement. Over the follow-up period, no ocular or periocular deformities or complications developed in these patients. CONCLUSION: Patients suspected of CL should be evaluated and treated early in the course of their disease to prevent any permanent ocular or periocular deformities.     

红斑狼疮皮损中CD4^＋和CD8^＋T细胞的表达

目的检测红斑狼疮皮损区T淋巴细胞亚群CD4 和CD8 T细胞的表达情况。方法采用免疫组织化学法对红斑狼疮皮损区淋巴细胞进行检测,并与正常皮肤作对照。结果红斑狼疮患者皮损CD4 T细胞百分数明显高于正常对照组。CD8 T细胞百分数明显低于正常对照组。结论红斑狼疮患者可能存在CD4 T细胞活性增强,CD8 T细胞功能抑制,从而导致免疫紊乱而发病。