A possible mechanism for phenol-induced pemphigus

A possible mechanism for phenol-induced pemphigus lesions in genetically predisposed individuals is proposed that accounts for in vitro observations and cases of biochemical acantholysis, as well as the in vivo acantholysis in pemphigus induced by phenols. The mechanism involves the induction of interleukin-1a and tumor necrosis factor-a release by keratinocytes. These cytokines in turn have been shown to be involved in the regulation and synthesis of complement and proteases like plasminogen activator, which have been implicated in the pathogenesis of acantholysis in pemphigus vulgaris.     

Pemphigus Vegetans Occurring in a Split-Thickness Skin Graft

BACKGROUND: Pemphigus vegetans, a variant of pemphigus vulgaris, most commonly occurs in the flexural area. OBJECTIVE: To describe an unusual case of pemphigus vegetans occurring in a skin graft recipient site and to discuss the possible etiology. METHODS: We present a 41-year-old man who developed vegetating plaques from the graft recipient site of his left leg for 8 months. RESULTS: Based on the histopathologic findings of a skin biopsy, this case was diagnosed as pemphigus vegetans. The patient's condition was successfully treated with systemic corticosteroids and acitretin. CONCLUSION: Our case is unique in its presentation of pemphigus vegetans shortly after a split-thickness skin graft. Physicians should be aware of this entity while differentiating cutaneous lesions arising from a skin graft.     

Platelet‐rich plasma for resistant oral erosions of pemphigus vulgaris: A pilot study

Oral erosions and ulcers of pemphigus vulgaris (PV) are a debilitating condition that is usually difficult to treat. The wound healing properties of platelet‐rich plasma (PRP) encouraged us to evaluate its usefulness in treatment of non‐healing oral PV lesions. Seven patients with chronic oral PV, resistant to conventional therapy, were treated with weekly to monthly injections of PRP of affected mucosal membranes. All recruits reported improvement in pain and mastication and 6 of 7 patients had an improvement in pemphigus disease area index scores with PRP treatment. PRP injections seems to accelerate the healing process and decrease the pain and eating discomfort associated with the oral erosions and ulcers induced by PV.     

Low-level laser therapy on the treatment of oral and cutaneous pemphigus vulgaris: case report

Pemphigus vulgaris is a chronic autoimmune mucocutaneous disease that initially is manifested by painful intraoral erosions and ulcers which spread to other mucosa and the skin, generally more than 5 months after oral lesion manifestation. The treatment consists of prednisone alone or in combination with an immunosuppressive agent, and the clinical response is perceived within 2 to 4 weeks. Low-level laser therapy has been effective in accelerating the healing of injured tissue, thus inducing cell proliferation and increasing ATP, nucleic acid, and collagen synthesis. We reported two cases of pemphigus vulgaris that received systemic treatment associated with low-level laser therapy for oral and cutaneous lesions. We observed prompt analgesic effect in oral lesions and accelerated healing of oral and cutaneous wounds. Therefore, the present report suggests LLLT as a noninvasive technique that should be considered as an adjuvant therapy in oral and skin disorders in patients with PV.     

Anaesthetic management of a patient with pemphigus vulgaris for emergency laparotomy

A 45-year-old man with a long-standing history of duodenal ulcer presented with symptoms and signs of perforation peritonitis. He also had lesions of pemphigus vulgaris throughout the body, involving both skin and mucous membranes. Care was taken to avoid pressure and friction during placement of monitoring devices, intravenous and arterial lines. Since the patient had to undergo exploratory laparotomy, intubation was performed in an atraumatic manner after rapid sequence induction. However, there was minor bleeding from the mucous lesions of the oral cavity, which was controlled by a saline adrenaline throat pack. The patient was extubated at the end of the surgery and steroids were continued in the peri-operative period.     

Delayed and localized pemphigus vulgaris after breast cancer radiotherapy

Breast cancer treatment involving ionizing radiation causes characteristic radiation dermatitis in the majority of patients. The DNA damaging effects of radiation can rarely predispose to primary inflammatory dermatoses, such as pemphigus vulgaris. In such cases, the disease presents with all the hallmarks of the primary dermatosis, but the eruption is limited to the field of irradiation and is often amenable to treatment. In contrast, occurrence of generalized pemphigus vulgaris in this setting may mean cancer recurrence. The mechanism by which radiotherapy induces localized disease remains unknown, but there is likely a loss of self‐tolerance which maybe coupled to antigen exposure.     

Pemphigus vulgaris presenting as chronic osteomyelitis of the foot

We describe a very unusual case of pemphigus vulgaris presenting as unilateral foot ulceration which was thought to be clinically chronic osteomyelitis. Only one other similar case has been published in the English language, describing a 60-year-old Malay male. Our case is an 81-year-old lady with a 2-week history of ulceration of the right forefoot. X-ray interpretation and clinical findings were thought to be consistent with osteomyelitis by the surgeons, and she went on to have amputation of the great toe. Referral to the Dermatology Department was made on the development of a new erosion on her left breast, whence clinical, histological and immunological findings were then consistent with pemphigus vulgaris.     

Pemphigus vulgaris and pregnancy

The management and counseling of patients with pemphigus vulgaris during pregnancy is a challenge. The frequency of the association is very low and the current knowledge is based only on case reports or small series. The authors report 2 cases ofpemphigus vulgaris and pregnancy that differed from each other in the time of occurrence and clinical course but had similar favorable outcomes. Based on a literature review and their personal observations, the authors discuss the characteristics of this association, the therapeutic behavior, patients' followup, and fetal prognosis.     

Tracheal mucosal bulla found on tracheal extubation in a patient with pemphigus vulgaris--a case report

A 49-year-old female with pemphigus vulgaris underwent the removal of a meningioma under general anesthesia. Neither bulla nor erosion was observed on her skin and oral cavity mucosa. She had been on prednisolone 15 mg for six years daily to avoid the recurrence of skin lesion. Anesthesia was induced and maintained with total intravenous anesthesia with propofol and fentanyl. No adverse episodes were encountered during the operative procedure. We checked the tracheal mucosa using bronchofiberscope before extubation. A small bulla was found on the tracheal mucosa, where the cuff of the tracheal tube was located. The trachea was extubated slowly under bronchofiberscopic observation, and no other bullae were found. It would have been formed by mechanical stimulation of the tracheal tube. This case suggests that we have to pay careful attention to the formation of bullae at any part of the body by mechanical stimuli during anesthetic management of patients with pemphigus vulgaris.     

Polymyositis and pemphigus vulgaris in a patient: successful treatment with rituximab

Rituximab has been documented to be an effective treatment for autoimmune diseases with contribution of B cells. We report a case of antisynthetase syndrome with a history of EBV-induced lymphoma which developed a pemphigus vulgaris. Rituximab was effective both on polymyositis and on pemphigus. Fifteen months later, the patient died from a septic shock after the first cyclophosphamide infusion for amyloidosis while the B cell population remained depleted. Rituximab may be a good alternative to immunosuppressive drugs in polymyositis and pemphigus especially in lymphoma-risk patients. However, it did not prevent progression to secondary amyloidosis and the fatal infection developed in this patient raises the question of whether a prolonged B cell depletion with rituximab contributes to a greater risk of infection.     

Pemphigus vulgaris triggered by rifampin and emotional stress

A 56-year-old Jewish woman of Ashkenazi origin had a history of tuberculosis since the age of 1 year, contracted while she was in hiding in Poland during the Holocaust. She developed bronchiectasis in 1972 for which she was being treated 4 times a year for 1-month periods with 600 mg/d rifampin. During the Persian Gulf War in February 1991, a month after a rifampin treatment, she was admitted to the dermatology department with cutaneous and oral mucous eruptions. Physical examination revealed crusting bullae and erosions on the trunk and lower limbs. The mucous membranes were affected in the vagina, oral cavity, and pharynx. Histologic and immunofluorescence studies on a biopsy specimen confirmed the diagnosis of pemphigus vulgaris. There was no family history of the disease. Systemic treatment with prednisone,100 mg/d, resulted in regression of the eruption within a month, and the dose was tapered to 10 mg every second day. Although she received anti-tuberculosis treatment, the steroids caused an exacerbation of the tuberculosis, and after a massive hemoptysis in 1993, a right upper and middle lobectomy was performed. The operation put an end to the vicious cycle of drug-induced pemphigus outbreaks by eliminating the need for rifampin, but the patient reported the appearance of new lesions during periods of emotional stress.     

The significance of desmosomes in pathology

Considerable progress has been made in our knowledge of desmosomes and their components. Molecular cloning of the desmosomal glycoproteins has established that desmoglein 1 and desmoglein 3 are targets for autoantibodies in the blistering diseases pemphigus foliaceus and pemphigus vulgaris respectively. New evidence suggests that another desmosomal glycoprotein, desmocollin 1, is the major target antigen in the upper epidermal form of intercellular IgA dermatosis. In human cancer there is accumulating evidence which suggests a role for desmosomes in the prevention of the invasion and metastasis. The possibility exists that a mutation in a desmosomal glycoprotein gene is responsible for an inheritable human disease, the striated form of palmoplantar keratoderma.     

Pemphigus vulgaris mimicking Paget's disease of the breast

We report the development of an ulcerative skin lesion involving the areola of a 37-year-old woman. Clinically the lesion was compatible with Paget's disease, however, histologic evaluation identified pemphigus vulgaris. Differences in the presentation and treatment of these two entities are highlighted. The underlying role of plasminogen activator in the molecular pathology of both diseases is discussed.     

Minimal change nephropathy and pemphigus vulgaris associated with penicillamine treatment of rheumatoid arthritis

A 51-year-old man developed pemphigus vulgaris, minimal change nephropathy and acute tubular necrosis after 11 months of treatment with penicillamine for rheumatoid arthritis associated with features of Reiter's syndrome. Penicillamine is a polyclonal B lymphocyte activator in animals and precipitates a variety of autoimmune reactions in man. The pathogenesis of minimal change nephropathy is obscure, but our observations support the possibility that autoimmune mechanisms play a role.     

Pemphigus with thymoma improved by thymectomy: report of a case

A 53-year-old female with pemphigus vulgaris received treatment with prednisolone for 3 years. On chest computed tomography performed at follow-up, an anterior–mediastinal tumor (4 cm × 3 cm) was detected and diagnosed as a thymoma. Although amyosthenia was absent, the patient’s anti-acetylcholine-receptor antibody level was high, and she was positive for anti-desmoglein 3 antibodies. She underwent extended thymectomy in the same year, following which both the anti-acetylcholine receptor antibody and the anti-desmoglein 3 antibody levels were normalized. The patient’s skin symptoms improved, and the steroid dose was gradually lowered and finally discontinued 4 years postoperatively. Extended thymectomy may be an effective therapy for treating patients with pemphigus.     

Gastric Cancer and Paraneoplastic Pemphigus

Paraneoplastic pemphigus is a relatively rare but significant acquired autoimmune mucocutaneous disorder that is characterised by diffuse erythema, painful blistering and sores of the skin and mucus membranes. The underlying pathogenesis is believed to be triggered by altered immune system in response to underlying neoplasm. The manifestations can predate, occur at the same time or after the diagnosis of cancer. Associations with gastric cancer have only been reported twice. A 78-year-old lady presented with a month’s history of extensive skin lesions that started off as bullous lesions and biopsy revealed bullous pemphigus. Endoscopy for anemia revealed gastric cancer. This case reinforced the need to consider underlying malignancy in elderly patient with new onset dermatological presentation.     

强脉冲光美容效果的评价

目的观察强脉冲光对皮肤表浅色素性和血管性疾病、寻常痤疮的疗效;评价其嫩肤美容及脱毛的疗效。方法采用GP666型强脉冲比（IPL）治疗仪对皮肤老化、面部色沉性皮损、皮肤毛细血管扩张、寻常痤疮及多毛症各20例共100例进行治疗,光子嫩肤每3周治疗1次,5次为1个疗程;脱毛每月治疗1次。结果经5次治疗,色素痫和血管痫有效率为90％,痤疮疗效为75%,美容效果为100％,脱毛效果为90％。无小可逆不良反膻发牛。结论IPL能有效治疗皮肤浅表色素沉着性皮损和毛细血管扩张;有较好的关容效果;脱毛效果肯定。     

Anaesthetic considerations in Castleman's disease

Castleman's disease is a lymphoreticular disorder, often localized and clinically silent. Systemic manifestations may occur. Castleman's disease may be associated with pathologies such as POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes), AIDS, amyloidosis, pemphigus vulgaris, and other types of cancer such as lymphoma and Kaposi's sarcoma. Careful preoperative evaluation is required to determine whether associated mediastinal or spinal canal masses or haematological disorders are present. The significance of these pathologies for anaesthetic management is discussed. We present the case of a 27-year-old female patient with Castleman's disease (hyaline-vascular variant) requiring vaginal reconstruction due to recurrent sores and synechiae.     

腹部局限性Castleman病副肿瘤性天疱疮八例临床分析

目的 探讨腹部局限性Castleman病副肿瘤性天疱疮的临床表现和临床治疗方法。方法 对我院收治的8例伴有腹部局限性Castleman病的副肿瘤性天疱疮患者的临床资料进行分析,探讨该病的临床和病理特征,总结其外科治疗效果。结果 8例患者均有典型天疱疮皮肤损害,伴有难治性口唇黏膜溃疡、糜烂;病理改变表现为表皮内疱、基底细胞液化变性,表皮内散在坏死的角质形成细胞及真皮浅层以淋巴细胞浸润为主。8例患者CT检查均发现内脏单发肿瘤。5例出现阻塞性细支气管炎。所有患者常规激素治疗无效,肿瘤切除后药物治疗1～2个月皮肤及黏膜损害治愈,肺部病变缓解,切除的肿瘤位于腹膜后,病理改变符合Castleman病。结论 腹部局限性Castleman病副肿瘤性天疱疮是一罕见疾病,发现和切除合并的内脏肿瘤是治疗的关键。     

高强度窄谱蓝光治疗面部寻常性痤疮临床观察

目的:观察高强度窄谱蓝光治疗面部寻常性痤疮的疗效。方法:应用波长为405～420nm的高强度窄谱蓝光治疗93例面部寻常性痤疮患者,每周2次,共治疗8次。计数患者炎性皮损(丘疹、脓疱、结节和囊肿)、非炎性皮损(粉刺)及皮损总数来计算改善率;观察患者治疗前后皮肤黑素指数、红斑指数、皮脂分泌及角质层含水量的变化情况。结果:治疗结束时,总有效率为82.80%。非炎性及炎性皮损平均改善率分别为51.67%和68.90%;患者面部红斑指数及皮脂分泌显著降低,而黑素指数及角质层含水量与治疗前比较无显著变化。随访3个月,总有效率与治疗结束时相比差异无统计学意义。结论:高强度窄谱蓝光治疗面部寻常性痤疮疗效显著,且可以减轻皮肤红斑、减少皮脂分泌。