Dyshidrosiform pemphigoid: report of a case

A Japanese woman had bullous eruptions on her palms and soles for two months, histopathologic examination, immunofluorescence microscopy, and ELISA for BP180 antigen led to the definitive diagnosis of dyshidrosiform pemphigoid (DP). This paper also includes a study of 15 other cases of DP.     

Localized pemphigoid simulating dyshidrosiform dermatitis.

A patient had a localized, persistent vesicular eruption of the feet that was clinically identical to dyshidrosiform dermatitis. Direct and indirect immunofluorescent studies revealed a bullous pemphigoid (BP) pattern. Antibody blocking experiments showed the patient's circulating basement membrane zone antibody to be identical to that of classical BP. Possible pathogenetic mechanisms of localized BP are discussed.     

Bullous pemphigoid—a report of 86 cases from Taiwan

We reviewed 86 cases of bullous pemphigoid and the results were compared with those reported in rhc literature. Seventy-eight per cent of the patients developed generalized blisters and 22% hntl localized blisters, including three cases of dyshidrosiform pemphigoid and one case of pretibial pemphigoid. Oral mucosal involvement was noticed in 12.8% of the patients, Fifteen per cent of the patients had internal malignancies but the incidence was not significantly different from the control group. Direct immunofluorescence in our series showed a high positive rate of 98-8%. Indirect imniunofluorescenee was positive in 48.1% of the 54 patients in whom this was carried out. Peripheral blood eusinophilia was observed in 221% of rhe patients. Prednisolone alone or in combination with immunosuppressive agents was the mainstay of treatment. Treatment side-effects was observed in 33% of the patients. Thirty percent of the patients had a complete remission after a mean follow-up period of 269 months.     

Two cases of dyshidrosiform pemphigoid with different presentations

We report two cases of dyshidrosiform pemphigoid (DP) with different presentations. One patient was a 65-year-old Japanese man, who had been diagnosed with dyshidrosis and had been treated before visiting our hospital. When we stopped all treatments, the vesicles increased and spread to the trunk and limbs. We made a diagnosis of vesicular pemphigoid (VP) that was concomitant with or transformed from DP. Using Western blotting, the sera reacted with antigens with molecular weights of 60 and 180 kDa. The 60-kDa antigen has not been found previously in the sera of patients with VP. The other patient was a 94-year-old Japanese woman, who presented with redness and swelling with bullae on the palmoplantar areas. Five days later, areas of oedematous erythema, as seen in prototypical bullous pemphigoid (BP), developed on the limbs. Study of the distribution of the BP antigen may elucidate the mechanisms involved in localized forms of BP such as DP.     

Western blot analysis of the antigen in pemphigoid gestationis

Using an immunoblotting technique, sera from 25 patients with pemphigoid gestationis were examined and tested against epidermal and dermal extracts of normal skin. The major antigen recognized by seven patients' sera was a molecule of 180 kDa, pemphigoid gestationis antigen, extractable only from the epidermis. Sera from 18 patients with bullous pemphigoid were studied as positive controls and the major antigen recognized was a larger molecule of 220 kDa. There was some degree of shared recognition of antigens with three patients with pemphigoid gestationis recognizing the 220 kDa bullous pemphigoid antigen. In addition one bullous pemphigoid serum recognized the 180 kDa pemphigoid gestationis antigen. The dominant pemphigoid gestationis antigen, however, differs from bullous pemphigoid antigen.     

Autoantibodies to bullous pemphigoid antigen 180 induce dermal-epidermal separation in cryosections of human skin

Bullous pemphigoid is a subepidermal autoimmune blistering disease associated with autoantibodies to the hemidesmosomal bullous pemphigoid antigens 180 and 230. Most sera from bullous pemphigoid patients recognize epitopes within the N-terminal NC16A portion of the bullous pemphigoid 180 ectodomain. Using cryosections of human skin, patients' sera were shown to generate dermal-epidermal separation when coincubated with leukocytes and complement from healthy volunteers; however, the specificity of pathogenic autoantibodies in bullous pemphigoid patients has not yet been elucidated. In this study, by the use of a modified version of the cryosection model, we show that sera from all of 13 bullous pemphigoid patients and from two rabbits, immunized against bullous pemphigoid 180 NC16A, induced dermal-epidermal separation. This finding was confirmed with the use of IgG purified from patients' sera, whereas sera and purified IgG from healthy controls were not pathogenic. The induction of subepidermal splits in this experimental model was shown to be dependent on the presence of neutrophils, but not complement. Interestingly, patients' autoantibodies affinity purified against a recombinant form of bullous pemphigoid 180 NC16A retained their blister-inducing capacity, whereas patients' IgG depleted of reactivity to NC16A lost this ability. F(ab')2 fragments of antibodies specific to NC16A, lacking the Fc portion, did not induce splits. In addition, patients' autoantibodies purified against a recombinant fragment of the C-terminus of bullous pemphigoid 180 as well as rabbit antibodies to the intracellular portion of bullous pemphigoid 180 and to bullous pemphigoid 230 did not cause dermal-epidermal separation. Our in vitro results support the idea that autoantibodies to bullous pemphigoid 180 from patients with bullous pemphigoid are of pathogenic relevance.     

Bullous pemphigoid and multiple sclerosis: more than a coincidence? Report of three cases

In three patients with long-standing multiple sclerosis, bullous pemphigoid developed. The diagnosis of bullous pemphigoid was based on histologic findings, direct and indirect immunofluorescence, and Western blots showing IgG reacting with the 220 to 240 kD bullous pemphigoid antigen in the serum of three patients. Contrary to previous observations, bullous pemphigoid associated with multiple sclerosis was not different from bullous pemphigoid alone. Three similar cases have been reported previously, so the occurrence of bullous pemphigoid in patients with multiple sclerosis may be more than a coincidence.     

Localized bullous pemphigoid: a commonly delayed diagnosis

Localized bullous pemphigoid is a rare clinical variant of bullous pemphigoid. We report three cases of localized bullous pemphigoid in which the diagnosis was delayed because the presentation simulated other local vesicular or bullous diseases. Localized bullous pemphigoid differs from the generalized form by its more benign course. Early diagnosis will help avoid unnecessary and costly treatment for unrelated conditions.     

Localized bullous pemphigoid induced by thermal burn

BACKGROUND: Induced bullous pemphigoid is known to be triggered by drugs and some physical agents. Six cases of bullous pemphigoid induced by thermal burns have been reported since 1991. CASE REPORT: We observed a bullous eruption around thermal burns of the left leg in a 89 year-old woman. Bullous pemphigoid was diagnosed by histological and immunohistochemical findings with a sub-epidermal blister and linear IgG and C3 deposition at the dermal-epidermal junction. Indirect immunofluorescence was negative. The eruption rapidly resolved with topical steroids. DISCUSSION: We describe a bullous pemphigoid induced by a thermal burn. This case is original because the eruption was localized only around the site of the burn and healed with topical steroids. In the literature, there are 6 other cases reported but only one localized. We discuss the hypothesis of asymptomatic bullous pemphigoid exacerbated by presentation of bullous pemphigoid antigen by thermal burns or self immunization against antigens altered by burns.     

大疱性类天疱疮患者发根毛囊中抗体的检测

目的：通过检测大疱性类天疱疮患者拔出的头发上残留毛囊中大疱性类天疱疮抗体的沉积情况,建立一种快捷、损伤小的检测大疱性类天疱疮的方法。方法：直接免疫荧光方法测定35例大疱性类天疱疮患者拔出的头发上残留毛囊中类天疱疮抗体的沉积,10例健康人及20例天疱疮病人头发作为对照。结果：35例大疱性类天疱疮患者的毛发,其中阳性28例,阴性7例,阳性率80%;类天疱疮IgG和C3在毛囊表皮下的外毛根鞘外层与结缔组织鞘之间呈明显的线状沉积。10例正常人及20例天疱疮患者均无线状沉积。结论：本实验方法简单、快速、敏感,且具有较高特异性,有可能成为诊断和鉴别诊断大疱性类天疱疮的一种有效的方法。     

Bullous pemphigoid. Occurrence in psoriasis treated with psoralens plus long-wave ultraviolet radiation.

The occurrence of bullous pemphigoid during treatment for psoriasis has been described in the literature. We saw a case of psoriasis that was complicated by bullous pemphigoid in a patient receiving orally administered psoralen followed by long-wave ultraviolet radiation (PUVA) therapy. In our patient the localization of bullous lesions to psoriatic plaques demonstrates that the PUVA-treated psoriatic skin may have a decreased threshold for the development of clinical bullous pemphigoid. This suggests factors other than simple coexistence of the two diseases. Therapy with PUVA might induce bullous pemphigoid in psoriasis, either primarily or by facilitating the expression of a previously existing subclinical form of the bullous disorder.     

Psoriasis and bullous pemphigoid

The coexistence of psoriasis and bullous pemphigoid in 44 patients has been described in the literature. In the majority of cases no causative agent was found for the development of bullous pemphigoid, but in many cases the bullous eruption was related to antipsoriatic treatment such as PUVA, anthralin, tar and salicylic acid. A 46-year-old man with a 20-year history of psoriasis who developed bullous pemphigoid without any previous therapy is described. After clearing, a later exacerbation of both psoriasis and bullous pemphigoid indicated that there is a pathogenetic relationship between the diseases. Among 72 patients with bullous pemphigoid no others suffered from psoriasis.     

Bullöse Skabies und durch Skabiesbefall getriggertes bullöses Pemphigoid

Two patients were admitted to our hospital with tense blisters on an erythematous base, typical for bullous pemphigoid. In both patients an infestation with Sarcoptes scabiei was diagnosed by dermatoscopy as well as histological examination. In one patient the clinical diagnosis of bullous pemphigoid could be confirmed by immunofluorescence microscopy, histopathology and a clinical relapse of bullous pemphigoid without scabies infestation. In the other patient no evidence for an autoantibody-mediated autoimmune blistering disease was found.We postulate that bullous scabies could develop after long persistency of the parasites leading to a specific immune response with activation of T helper type 2 (Th2) cells causing high levels of the cytokine interleukin 5 and then consecutively eosinophilia. Secretion of proteolytic enzymes near the basal membrane zone might explain the development of intraepidermal, often suprabasal blisters. In contrast, in the first patient the scabies infestation might have triggered a flare up of the underlying autoimmune disease. Comparison of our two patients demonstrates two entities: bullous pemphigoid triggered by scabies as a Koebner phenomenon and a bullous subtype of scabies mimicking bullous pemphigoid.Therefore both, scabies infestation triggering bullous pemphigoid and bullous pemphigoid-like scabies should be included in the differential diagnosis of vesicles, tense blisters and erythema, especially at an early clinical stage.     

Concomitant psoriasis and bullous pemphigoid: coincidence or pathogenic relationship?

Psoriasis vulgaris and bullous pemphigoid represent two clinically well-characterized, chronic, inflammatory skin diseases. The concomitant occurrence of these two entities in a patient is rare, and the pathogenic implications of this phenomenon are unknown. We describe a 55-year-old woman with a 25-year history of plaque-type psoriasis who presented with disseminated tense bullae. Skin biopsies showed the typical histologic and immunohistochemical traits of bullous pemphigoid, and she had circulating immunoglobulin G (IgG) antibodies against the basement membrane zone, specifically the BP180 antigen. The bullous eruption was successfully treated with oral methylprednisolone and dapsone. Bullous pemphigoid is the autoimmune blistering disease that has most often been associated with psoriasis. Forty cases have been described in the literature. Classical psoriasis and psoriasis associated with bullous pemphigoid are identical. The pathogenic relationship between psoriasis and bullous pemphigoid is unclear. It has been postulated that the autoimmune process responsible for bullous pemphigoid lesions may be induced by ultraviolet light therapy, topical corticosteroids, and/or the inflammatory processes that occur in psoriasis. Immunomodulatory therapy may positively influence shared as well as distinct inflammatory mechanisms in patients who have psoriasis and bullous pemphigoid.     

Coexistence of pemphigus foliaceus and bullous pemphigoid. Demonstration of autoantibodies that bind to both the pemphigus foliaceus antigen complex and the bullous pemphigoid antigen

Pemphigus and bullous pemphigoid are autoimmune blistering diseases of the skin characterized by circulating autoantibodies directed against the keratinocyte cell surface and the epidermal basement membrane zone, respectively. The coexistence of pemphigus and bullous pemphigoid is very uncommon. We describe a patient with pemphigus foliaceus who later developed bullous pemphigoid and show, by means of immunoprecipitation studies utilizing both cultured keratinocytes and suction blister epidermis, that our patient had circulating autoantibodies directed against both the pemphigus foliaceus antigen complex and the bullous pemphigoid antigen. This report is the first to demonstrate the coexistence of pemphigus foliaceus and bullous pemphigoid at the molecular level.     

Antibasement Membrane Zone Antibodies In Localized Pretibial Pemphigoid

To clarify the nosologic position of localized pretibial pemphigoid, Western immunoblotting analysis was carried out using the sodium dodecyl sulfate extracts of normal human epidermis. Sera from patients with localized pretibial pemphigoid and generalized bullous pemphigoid reacted with 220- to 240-kd polypeptide, which is a critical point for a definite diagnosis of bullous pemphigoid. This result suggests that localized pretibial pemphigoid belongs to the same nosologic position as bullous pemphigoid.     

Localized bullous pemphigoid at urostomy site

Localized bullous pemphigoid occurs in less than one-third of the cases of bullous pemphigoid and it usually appears on the shins. Localized bullous pemphigoid around a stoma site is very uncommon, with few reports in the literature. We report a case of localized bullous pemphigoid at urostomy site and we review its main characteristics. We highlight the importance of considering bullous pemphigoid in chronic bullous lesions on peristomal skin.     

Dual diagnosis of Pemphigus and pemphigoid. Retrospective review of thirty cases in the literature.

BACKGROUND: Pemphigus and pemphigoid are two distinct groups of autoimmune blistering diseases. There are many reports of the simultaneous presence of clinical and serological features of both diseases in the same patient. OBJECTIVE: This study is a retrospective review of the present literature on reports of patients with features of both pemphigus and pemphigoid. We recommend that these patients be considered as having a dual diagnosis. METHODS: A review of the English language, peer-reviewed literature was conducted on patients described with features of pemphigus and pemphigoid. Available data on clinical profile, histology, immunopathology, treatment, follow-up and outcome were studied in 30 patients. They were divided into three groups: (1) bullous pemphigoid and pemphigus vulgaris, (2) mucous membrane or cicatricial pemphigoid and pemphigus vulgaris and (3) bullous pemphigoid and pemphigus foliaceus. RESULTS: In all three groups, most patients had a clinical phenotype resembling both diseases. In 17 patients with bullous pemphigoid and pemphigus vulgaris, 83% had a skin biopsy consistent with bullous pemphigoid, 70% had direct immunofluorescence studies typical of bullous pemphigoid and sera of 83% had antibodies typical of pemphigus vulgaris on indirect immunofluorescence. In 10 patients with mucous membrane or cicatricial pemphigoid and pemphigus vulgaris, a histology of mucous membrane pemphigoid was reported in 60% of the patients, direct immunofluorescence studies typical of mucous membrane pemphigoid were reported in 70% of the patients and in 80%, autoantibodies characteristic of pemphigus vulgaris were observed. In 3 patients with bullous pemphigoid and pemphigus foliaceus, the histologies were consistent with bullous pemphigoid, direct immunofluorescence was typical of pemphigus foliaceus and their sera had both autoantibodies. The majority of the 30 patients required long-term high-dose corticosteroids and immunosuppressive agents to control their disease. Three patients with bullous pemphigoid and pemphigus vulgaris (18%) died due to effects of prolonged immunosuppression. CONCLUSION: We characterize a group of patients who have clinical, histological and immunopathological features of bullous or mucous membrane or cicatricial pemphigoid with serological features of pemphigus. These patients did not achieve a prolonged clinical remission by conventional therapy. It is possible that early identification of these patients may improve their prognosis.     

Bullous pemphigoid evolving into cicatricial pemphigoid?

We describe three patients who initially presented with both clinical and immunological findings to support a diagnosis of bullous pemphigoid but whose subsequent course has been that of cicatricial pemphigoid. Mucosal scarring was accompanied by a fall in autoantibody titres in our three patients. These cases illustrate the difficulties clinicians may experience in assigning a specific diagnosis to patients. They also support the concept that bullous pemphigoid and cicatricial pemphigoid are part of a single disease spectrum. The most intriguing question is what specific factors determine the expression of a particular disease phenotype as bullous pemphigoid and cicatricial pemphigoid share target antigens and also the DQ7 allele.     

10例寻常型银屑病合并大疱性类天疱疮临床分析

目的：总结寻常型银屑病合并大疱性类天疱疮患者的临床特征及治疗方法。方法：回顾性分析2016年1月至2018年12月我院住院患者中诊断为寻常型银屑病合并大疱性类天疱疮的临床资料。结果：共发现10例寻常型银屑病合并大疱性类天疱疮患者,其中男9例,女1例,平均年龄为（59.90±10.18）岁,银屑病均先于大疱性类天疱疮发病。8例患者水疱发生于银屑病斑块上,2例水疱发生于外观正常的皮肤上。9例患者采用糖皮质激素和/或免疫抑制剂治疗,1例患者应用口服四环素、烟酰胺配合光疗治疗。出院后随访150~1065天,7例患者无新发水疱,银屑病皮损病情稳定;2例失访,1例死亡。结论：寻常型银屑病合并大疱性类天疱疮临床相对少见,中老年患者居多,糖皮质激素联合免疫抑制剂治疗有效。