Mohs Micrographic Surgery-Induced Pemphigus

BACKGROUND: Pemphigus is an autoimmune blistering disease that presents with flaccid intraepidermal blisters, erosions of the skin and mucous membranes, acantholysis, and in vivo bound and circulating autoantibodies against keratinocyte antigen. Currently a handful of reports incriminate surgical trauma as an initiating factor in this disease. OBJECTIVE: To document pemphigus evolving in a wound after Mohs micrographic surgery. METHODS: Case report. RESULTS: We present a case of pemphigus that started in a Mohs surgical wound after the excision of a squamous cell carcinoma (SCC) from a 49-year-old woman. Biopsy of the preoperative lesion did not reveal pemphigus. Biopsy of the postoperative lesion revealed pemphigus with no residual SCC. CONCLUSION: We suggest that Mohs surgery, like any other skin surgery, may nonspecifically activate pemphigus. This change must be differentiated from postoperative wound infection and other causes of poor wound healing.     

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.     

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.     

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 Foliaceus Masquerading as Postoperative Wound Infection: Report of a Case and Review of the Koebner and Related Phenomenon following Surgical Procedures

BACKGROUND: The Koebner phenomenon, also known as the isomorphic response, is the development of preexisting skin disease following trauma to uninvolved skin. Various cutaneous disorders have been described to arise at surgical wounds and scars. Moreover, dermatologic procedures, such as cold-steel and laser surgery, can evoke koebnerization. OBJECTIVE: To describe a case of pemphigus foliaceus arising in postoperative wounds and to present a review of dermatologic disorders triggered by surgical procedures. METHODS: We report a case of pemphigus foliaceus initially presenting at sites of Mohs' micrographic surgery, shave biopsy, and cryotherapy and, subsequently, at a nonsurgical site. We reviewed the English literature in MEDLINE from November 1955 to April 2004 for reports of Koebner and related phenomenon following surgical procedures. RESULTS: To our knowledge, this is the first reported case of pemphigus foliaceus erupting at surgical and cryotherapy wounds. The clinical appearance can mimic wound infection. In addition to inducing preexisting disease, cutaneous procedures can also trigger the onset of new disease, which can either be limited only to the surgical site or subsequently become generalized. CONCLUSION: Postoperative Koebner or related responses should be included in the differential diagnosis of poorly healing surgical wounds. Skin biopsies for histopathology and immunologic studies may be necessary for definitive diagnosis and optimal management.     

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.     

Hidradenitis Suppurativa Associated with Galli-Galli Disease: Extending the Link with Dowling-Degos Disease

Galli-Galli disease (GGD) is a rare genodermatosis that is distinguished from Dowling-Degos disease (DDD) by the histologic finding of acantholysis. We present a case of a female patient with pruritic intertriginous plaques and history of hidradenitis suppurativa (HS). While reports exist associating DDD with HS, to our knowledge, GGD in association with HS has not been reported in recent literature. HS in association with DDD has been found to have causal mutations, involving the gamma-secretase complex and POFUT1 genes. DDD also has shared causal mutations with GGD in the POGLUT1 and KRT5 genes. These three skin diseases have been linked to different gene mutations, which are all associated with the Notch signaling pathway.     

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 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.     

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.     

Recalcitrant Hailey-Hailey Disease Successfully Treated with Low-dose Naltrexone

Hailey-Hailey (HHD), or benign familial chronic pemphigus disease, is a rare autosomal dominant blistering disorder characterized by recurrent vesicles that erode and macerate into weeping and crusting plaques. HHD has been shown to be resistant to several treatment options. Although not yet approved as a treatment for HHD, recent reports have suggested the use of low-dose naltrexone (LDN) as a successful treatment option for controlling recalcitrant HHD. We present a case of a 50-year-old woman with a 20-year history of biopsy-confirmed HHD with recurrent painful and pruritic vesicles and plaques. The patient developed significant clinical improvement of the cutaneous lesions with LDN treatment after only 26 days of treatment. It is important for dermatologists to consider LDN as a viable treatment option for HHD, especially in recalcitrant patients. We suggest this novel treatment as a rapidly effective option to resistant HHD.     

Pseudoangiosarcomatous squamous cell carcinoma: first case report on penis

Pseudoangiosarcomatous squamous cell carcinoma, also called pseudovascular, pseudoangiomatoid or adenoid pseudovascular carcinoma, is an uncommon and highly aggressive variant of squamous cell carcinoma. Histologically, it is characterized by proliferation of atypical keratinocytes with acantholysis and formation of pseudovascular spaces, forming anastomosed channels lined with neoplastic cells that invade the dermis. These cells are positive for cytokeratin and negative for vascular markers such as CD31 and CD34. There are few reports of this variant in the literature. Skin, breast, lung and vulva involvement have been described, but to the best of our knowledge, no cases involving the penis has been described. This study aims to describe the first case of angiosarcomatous squamous cell carcinoma of the penis. The patient presented with a painful lesion in the penis associated with urinary retention. Macroscopic findings exhibited an ulcerative vegetating lesion that involving the entire glans and part of the penile body, as well as infiltration of penile structures and scrotal skin. Microscopy shows atypical proliferation of sarcomatous keratinocyte pattern mimicking vascular spaces. Human papilloma virus (HPV) biomarkers and polymerase chain reaction (PCR) were all negative. Advanced penile squamous cell carcinoma with aggressive lymph node metastasis. This report presents the first case of penile pseudoangiosarcomatous squamous cell carcinoma, as an important differential diagnosis.     

Primary laryngeal and nasal septal lesions in pemphigus vulgaris.

Synchronous laryngeal and nasal septal lesions in pemphigus vulgaris are rare. The authors report a case of pemphigus vulgaris in an elderly man who was seen with lesions of the supraglottic larynx and nasal septum. The classic skin changes of pemphigus eventually developed, and the patient responded to daily treatments of 80 mg of prednisone. Steroids and immunosuppressive agents remain the treatment of choice, but a favorable prognosis is still dependent on early diagnosis and institution of therapy.     

Anesthesia for a patient with pemphigus erythematosus]

An 82-year-old female with pemphigus erythematosus had the head of femur replaced. In her case, systemic lupus erythematosus and myasthenia gravis, typical complications of pemphigus erythematosus, were not present. As even slight friction easily causes blisters in patients with pemphigus, we paid attention mainly to fragility of the skin and the mucus membranes and were careful to minimize physical stimulation of the skin. Because the mucosa of the pharynx or oral cavity could easily be damaged and form blisters during intubation, which in turn might make the management of the upper airway more difficult, we selected spinal anesthesia. No serious perioperative complications, except a few blisters around the wound were observed. We thus successfully managed anesthesia of a patient with pemphigus erythematosa.     

滤泡树突状细胞肉瘤并发副肿瘤性天疱疮1例并文献复习

目的报告1例滤泡树突状细胞肉瘤并发副肿瘤性天疱疮的临床表现及临床治疗,从而提高对该疾病多样临床表现的认识,避免误诊、漏诊的发生并完善相关诊治。方法分析1例滤泡树突状细胞肉瘤并发副肿瘤性天疱疮的临床表现及临床治疗并查阅国内外文献资料。结果此例是来自淋巴结外部的滤泡性树突状细胞肉瘤,因其细胞边界不清,明显的异型和有丝分裂而具有较高的肿瘤恶性程度,手术后患者的状态逐渐恶化。结论对于局限性病变,手术切除是首选治疗方式。但单纯手术具有较高的局部复发率,巩固性放疗可降低手术患者的局部复发率,并延长其无病生存期。对全身播散性FDCS(多处淋巴结肿大)、有巨大肿块或手术未能根治的患者,需要进行有效的联合化疗。     

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。     

HLA DQ region gene polymorphism associated with primary IgA nephropathy

IgA nephropathy (IgAN) has been associated with HLA-DR4. We have recently described two non-allelic Taq I DQ beta gene-associated fragments sized 2.0 kb (T2) and 6.0 kb (T6), which strongly associate with DR4. T2 represents a polymorphism of the DQ beta gene and has been redesignated DQw8 (10th International HLA Workshop). The origin of the T6 fragment has not been determined, but probably represents a polymorphism of either the DQ beta or DX beta gene. When present together T2 and T6 define a subgroup of DR4 subjects at high risk of developing autoimmune disease. We have, therefore, studied DQ beta gene polymorphisms in IgAN. The DR antigen distribution was similar in IgAN and normal controls. The T2+/T6+ phenotype was present in 49% patients with IgAN compared to 15% of controls [P less than 0.0001, chi 2 = 32.8, Cramer's V = 0.41; relative risk = 5.5 (range, 2.8-11.0)]. Seventy-two percent of DR4+ IgAN patients and 29% of DR4+ controls were T2+/T6+ (P = 0.007, chi 2 = 17.0). These findings confirm the hypothesis that disease susceptibility genes are important in IgAN, and suggest that the putative gene(s) are located within or near to the DQ subregion. Moreover, similar DQ beta gene associations have been found in IDDM and pemphigus vulgaris, pointing to a common immunogenetic mechanism predisposing to several autoimmune diseases.     

Castleman's disease associated with myasthenia gravis

Castleman's disease presents as a peculiar type of lymph node hyperplasia. Traditionally, the disease has been classified on clinical grounds (solitary or multicentric) and by histologic appearance (hyaline vascular pattern, plasma cell predominance, or mixed lesions). It is now increasingly clear that there are different etiologies for each of these different subtypes. Reported associations include POEMS syndrome (polyneuropathy, organomegally, endocrinopathy, monoclonal gammopathy, and skin changes), paraneoplastic pemphigus, Hodgkin's disease, and follicular dendritic cell sarcoma. We present a case of Castleman's disease associated with myasthenia gravis, the third reported case in the literature. We discuss Castleman's disease and review the literature.     

Acantholytic Squamous Cell Carcinoma in Upper Aerodigestive Tract: Histopathology, Immunohistochemical Profile and Epithelial Mesenchymal Transition Phenotype Change

Acantholytic squamous cell carcinoma is a rare variant of squamous cell carcinoma in the mucosa of upper aerodigestive tract. Histomorphologically, acantholytic squamous cell carcinoma may lose the typical features of conventional squamous cell carcinoma and mimic other epithelial or mesenchymal malignancies due to advanced acantholysis and dyskeratosis. Because of its rarity, information of prognosis, pathologic features and immunohistochemical profiles is limited. We have studied clinicopathologic features and immunohistochemical profiles of four acantholytic squamous cell carcinoma cases arising from upper aerodigestive tract. Clinical results indicate an aggressive biologic behavior. Morphologically, all tumors revealed significant acantholysis with separation of tumor cells and intratumoral spaces. The tumor cells were highly pleomorphic and growth patterns were variable. In immunohistochemical studies, all tumor cells revealed positive reactions for AE1/AE3 and p63 supporting a squamous epithelial origin. In contrast to conventional aerodigestive squamous cell carcinoma, acantholytic squamous cell carcinoma showed significant reductions of cytokeratin19, E-cadherin and concomitant up-regulation of vimentin expression. Both morphologic features and immunohistochemical profiles indicate that acantholytic squamous cell carcinoma has acquired an epithelial mesenchymal transition phenotype. However, in contrast to other solid malignant tumors, the epithelial mesenchymal transition phenotype change in acantholytic squamous cell carcinoma is not limited to the invasive front of the peripheral tumor but, rather, diffusely involves entire neoplastic lesion. In addition, because cytokeratin 19 staining is attenuated, this would be an insensitive marker for following up and/or in detecting disseminated tumor cells in cases of acantholytic squamous cell carcinoma in upper aerodigestive tract.     

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.