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Numerous mechanisms of action have been proposed for intravenous Ig (IVIG). In this study, we used IgG passive transfer murine models of bullous pemphigoid (BP), pemphigus foliaceus (PF), and pemphigus vulgaris (PV) to test the hypothesis that the effect of IVIG in autoantibody-mediated cutaneous bullous diseases is to accelerate the degradation of pathogenic IgG by saturation of the MHC-like Fc receptor neonatal Fc receptor (FcRn). BP, PF, and PV are organ-specific antibody-mediated diseases in which autoantibodies target the hemidesmosomal antigen BP180 and desmosomal antigens Dsg1 and Dsg3, respectively. Antibodies against BP180, Dsg1, and Dsg3, when injected into neonatal mice, induce the BP, PF, and PV disease phenotypes, respectively. We found that FcRn-deficient mice were resistant to experimental BP, PF, and PV. Circulating levels of pathogenic IgG in FcRn-deficient mice were significantly reduced compared with those in WT mice. Administration of high-dose human IgG (HDIG) to WT mice also drastically reduced circulating pathogenic IgG levels and prevented blistering. In FcRn-deficient mice, no additional protective effect with HDIG was realized. These data demonstrate that the therapeutic efficacy of HDIG treatment in the pemphigus and pemphigoid models is dependent on FcRn. Thus, FcRn is a promising therapeutic target for treating such IgG-mediated autoimmune diseases. [ABSTRACT FROM AUTHOR]

A pemphigus vulgaris (PV) patient with a 14-year history of severe and painful blistering of skin and mucous membranes as well as side effects from corticosteroids and concomitant immunosuppressive drug treatment was managed successfully by protein A immunoadsorption (IA). After 19 sessions of protein A IA, the patient showed remission of PV and healing of mucocutaneous lesions and the skin. The level of the pathogenic autoantibodies to the adhesion proteins desmoglein 1 (Dsg-1) and desmoglein 3 (Dsg-3) measured by ELISA and immunofluorescence microscopy revealed a significant removal of autoantibodies after each IA therapy. There was a weak rebound of anti-Dsg- 1 and anti-Dsg-3 antibodies between IA sessions but an overall decrease over the period of IA therapy. This case demonstrates the effective use of protein A IA as an adjuvant and corticosteroid-sparing therapy in severe pemphigus refractory to standard immunosuppressive therapy and underscores the need for careful monitoring of autoantibodies. [ABSTRACT FROM AUTHOR]

In pemphigus vulgaris, treatment with systemic glucocorticosteroids is life saving; it may, however, cause severe side effects, including death. A patient with pemphigus vulgaris and myasthenia gravis was treated for approximately five years with the cholinomimetic Mestinon (pyridostigmine bromide), Imuran (azathioprine), and a topical corticosteroid gel before the need to introduce systemic glucocorticosteroids. Because activation of keratinocyte acetylcholine receptors also has been shown to abolish pemphigus IgG–induced acantholysis in cultured keratinocyte monolayers, a clinical trial of Mestinon was initiated in patients with active pemphigus vulgaris, pemphigus foliaceus, and paraneoplastic autoimmune multiorgan syndrome (also known as paraneoplastic pemphigus). First results indicate that nonsteroidal treatment of pemphigus is possible. Mestinon may be used to slow down progression of the disease and to treat mild cases with chronic lesions on limited areas. Stimulation of the keratinocyte– acetylcholine axis may lead to a therapeutic effect through any of the following mechanisms: (1) stimulating keratinocyte cell-to-cell attachment; (2) accelerating re-epithelialization; and (3) competing with the disease-causing pemphigus antibodies, preventing them from attachment to keratinocytes. Glucocorticosteroids and various types of steroid-sparing drugs used to treat pemphigus exhibit cholinergic side effects, including effects on expression and function of keratinocyte adhesion molecules, that are very similar to those produced by the cholinomimetic drugs. Further elucidation of the mechanisms underlying therapeutic efficacy of antiacantholytics may shed light on the immunopharmacological mechanisms of pemphigus antibody–induced acantholysis. [ABSTRACT FROM AUTHOR]

Background: Pemphigus vulgaris (PV) is a severe antibody-mediated autoimmune blistering disease. Because some patients with PV do not enter into remission, despite the use of high-dose corticosteroid therapy and immunosuppressive adjuvant treatments, new effective and safer agents are warranted to treat refractory PV. Rituximab, a monoclonal anti-CD20 antibody, induces depletion of B cells in vivo and has shown efficacy in patients with refractory antibody-mediated autoimmune disorders. We describe herein 3 patients treated with rituximab for severe PV. Observations: Three patients with refractory PV were treated with rituximab, resulting in a clinical response in all patients, which was complete in 2 patients. A decline in titers of circulating antiepidermis autoantibodies paralleled disease activity, while circulating B cells remained undetectable for 6 to 10 months. Two patients experienced bacterial infection in the weeks following the rituximab course. A clinical relapse occurred in 2 patients, at 6 and 10 months. A second course of rituximab controlled the disease in one of them. Conclusion: These patients' response suggests that rituximab may be a valuable treatment for refractory PV and warrants further studies to evaluate the risk-benefit ratio in patients with PV showing resistance to classic therapy. [ABSTRACT FROM AUTHOR]

Background: Mycophenolate mofetil is increasingly being used as a corticosteroid-sparing agent in immunosuppressive regimens. Objective: To elucidate the effectiveness of mycophenolate as adjuvant therapy in the treatment of both pemphigus vulgaris and pemphigus foliaceus. Design: Historical prospective study. Setting: University hospital. Patients: The study included 42 consecutive patients with pemphigus (31 with pemphigus vulgaris and 11 with pemphigus foliaceus) who had relapses during predni-sone taper or had clinically significant adverse effects from previous drug therapy. Results: Remission was achieved in 22 (71%) and 5 (45%) of patients with pemphgus vulgaris and pemphigus foliaceus, respectively. Partial remission was achieved in 1 (3%) and 4 (36%), respectively. The median time to achieve complete remission was 9 months (range, 1-13 months). The treatment was administered for a median of 22 months, and the median follow-up period was 22 months. Seventy-seven percent of patients had no adverse effect. Two patients had side effects severe enough to necessitate discontinuation of treatment, one because of symptomatic but reversible neutropenia and the other because of nausea. Conclusion: Mycophenolate is an effective and safe adjuvant in the treatment of both pemphigus vulgaris and pemphigus foliaceus. [ABSTRACT FROM AUTHOR]

ABSTRACT A female patient with coexistence of pemphigus herpetiformis and systemic lupus erythematosus is described. She presented to our Department with pruritic vesicles on her trunk and extremities, which were later accompanied with butterfly like erythema on her face and with central nervous system (CNS) manifestations. The diagnosis of pemphigus herpetiformis was based on the clinical picture and immunofluorescence finding, because the histopathologic finding is not always typical for the diagnosis. The diagnosis of systemic lupus erythematosus was based on positive ANA and anti-dsDNA, presence of butterfly-like erythema on her face, and CNS manifestations. The patient was treated by corticosteroids in combination with immunosuppressants, which should ensure good control of both diseases. The coexistence of pemphigus herpetiformis and systemic lupus erythematosus has not been reported in recent literature. [ABSTRACT FROM AUTHOR]

Abstract Background Pemphigus vulgaris is a potentially life-threatening disease characterized by cutaneous and mucosal blistering. Systemic corticosteroids remain the mainstay of therapy, transforming an invariably fatal disease into one with a mortality that is now less than 10%. Nevertheless, oral lesions are often recalcitrant and corticosteroid therapy can provoke adverse effects. Objective To determine whether two different regimens of systemic corticosteroid therapy based on prednisone gave different benefits. Method We examined two different regimens of systemic corticosteroid therapy based on prednisone in an open study. Ten patients (group A) were treated with systemic corticosteroids, in a therapeutic protocol made up of orally administered prednisone. Ten matched patients (group B) were treated with systemic corticosteroids alternating a pulse of intravenous betamethasone with orally administered prednisone. Results and conclusions The pulse protocol appeared to have some advantages both in a shorter time to resolution of symptoms and oral lesions, and in terms of minor adverse effects. [ABSTRACT FROM AUTHOR]

Background There is growing evidence that immunoablative high-dose cyclophosphamide without stem cell rescue is effective and safe in patients with refractory autoimmune diseases such as paraneoplastic pemphigus, systemic lupus erythematosus, aplastic anemia, and more recently pemphigus vulgaris. Methods We report a 51-year-old patient with severe pemphigus foliaceus, which was recalcitrant to multiple medical regimes. The patient presented with multiple thick hyperpigmented and scaly, ill-defined plaques on the face. In addition, she had multiple superficial erosions and crusts on her scalp, thorax, upper and lower extremities. The patient also had a few discrete intact flaccid bullae. A skin biopsy and direct immunofluorescence was consistent with pemphigus foliaceus. The patient's circulating pemphigus autoantibodies were present at a titer of 1:2560. The patient received immunoablative high-dose cyclophosphamide (50 mg/kg/day) for 4 consecutive days, and tolerated the regime well. Results Approximately 3 months after therapy, the skin lesions had healed and her prednisone, which had been as high as 80 mg daily, was tapered to 30 mg daily. In addition, her circulating autoantibodies decreased after treatment. Nearly 10 months after treatment, the patient did relapse. However, her disease was less severe and more easily managed with lower doses of immunosuppressive therapy. Conclusion This case contributes to the growing evidence of high-dose cyclophosphamide's efficacy without stem cell rescue in recalcitrant autoimmune diseases, including pemphigus foliaceus. [ABSTRACT FROM AUTHOR]

Background Immunobullous diseases are uncommon in childhood. In contrast to adults, the most commonly seen is IgA-mediated chronic bullous disease of childhood (CBDC), while IgG-mediated bullous pemphigoid (BP), cicatricial pemphigoid (CP) and epidermolysis bullosa acquisita (EBA) are rare. We have demonstrated both IgG and IgA autoantibodies to basement membrane zone target antigens in eight children with ‘mixed immunobullous disease of childhood’. Objectives To elucidate whether a dual antibody response makes these patients distinct regarding their presentation, immunopathology, course and prognosis. Methods We compared the eight children showing the double antibody response with 62 children with CBDC, BP, CP and EBA in whom only one antibody isotype was demonstrated. Clinical information at presentation, clinical course and response to treatment were recorded, and immunoblotting and direct and indirect immunofluorescence (IF) were performed. Results Six of the eight patients presented with clinical features of CBDC. In two others, it was uncertain whether they had CBDC or BP. Seven of the eight demonstrated a dual antibody response on indirect IF and three on direct IF. Immunoblotting revealed a variety of epidermal and dermal target antigens (BP230, BP180, 97-kDa protein and laminin 5). Five of the eight responded well to dapsone, two to sulphonamides, and one to systemic erythromycin alone. The clinical course was not protracted. Five are in remission 1–4 years following treatment, and three still have active disease suppressed by treatment after 6 months-2 years. Conclusions Although we do not know why these children have ‘mixed immunobullous disease’ (the dual antibody response), our results indicate that the presence of IgA is associated with a good response to treatment with antimicrobials (dapsone, sulphonamides, erythromycin), and the clinical course is no more protracted than that found in children with a single... [ABSTRACT FROM AUTHOR]

Reports on the use of anti-CD20 monoclonal antibody (rituximab) for refractory erosive stomatitis secondary to CD20[sup +] follicular lymphoma-associated paraneoplastic pemphigus.

Objective: To compare the effectiveness and the adverse effects of 2 different regimens for the treatment of pemphigus: corticosteroids alone compared with a combination of corticosteroids and cyclosporine. Design: Concurrently randomized trial. Setting: Tertiary care medical center. Patients and Methods: We studied 33 sequential hospitalized patients with newly diagnosed pemphigus vulgaris (n = 29) or pemphigus foliaceous (n = 4) based on clinical, histological, and immunofluorescence criteria who had not previously been treated with systemic corticosteroids or immununosuppressive drugs. Patients were randomly assigned to treatment with methylprednisolone or prednisolone plus cyclosporine. Intervention: Both groups were treated with similar initial doses of prednisolone (prednisone equivalent, 1 mg/ kg), which were increased 50% every 5 to 10 days based on persistence of disease activity. One group was treated in addition with cyclosporine (5 mg/kg). Main Outcome Measures: Patients were followed up closely for clinical outcome based on time required to control active manifestations of the disease, induction of partial and complete remissions, total amount of corticosteroids administered, frequency of relapses, and development of complications. Results: The 2 groups were similar in terms of demographics and baseline disease severity. There was no difference between groups in any of the variables used to measure response to treatment or total amount of corticosteroids administered. Complications were more common in patients who received combination therapy. Conclusion: Combination treatment with corticosteroids and cyclosporine, 5 mg/kg, offers no advantage over treatment with corticosteroids alone in patients with pemphigus. [ABSTRACT FROM AUTHOR]

Pemphigus is a group of potentially fatal dermatoses with both cutaneous and oral manifestations. Characterized by the appearance of vesicle or bullae, their manifestations in the oral cavity often precede those on the skin by many months or may remain as the only symptoms of the disease. It is therefore important that the oral manifestations of the disease are recognized on time, to make a proper diagnosis and initiate timely treatment. Here we present a case of Pemphigus Vulgaris (PV) that presented with oral lesions at multiple sites including tongue, to highlight the importance of timely recognition of the oral lesions during routine dental practice for the diagnosis and management of this disease. [ABSTRACT FROM AUTHOR]

Of the autoimmune bullous diseases, pemphigus vulgaris is the most severe, requiring long-term immunosuppressive therapy and high doses. Although the disease can be controlled with therapy, severe complications can occur secondary to the immunosuppressive therapy. Pulmonary aspergillosis is a severe form of lung injury caused by Aspergillus spores that can appear on immunosuppressive status with life-threatening risk if not diagnosed and treated in time. We present a case of a patient with pemphigus vulgaris found in immunosuppressive therapy with systemic corticosteroids and azathioprine, who was diagnosed and treated for a severe form of pulmonary aspergillosis, occurred 4 months after the treatment of the underlying disease. [ABSTRACT FROM AUTHOR]

Pemphigus represents a group of rare and severe autoimmune intra-epidermal blistering diseases affecting the skin and mucous membranes. These painful and debilitating diseases are driven by the production of autoantibodies that are mainly directed against the desmosomal adhesion proteins, desmoglein 3 (Dsg3) and desmoglein 1 (Dsg1). The search to define underlying triggers for anti-Dsg-antibody production has revealed genetic, environmental, and possible vaccine-driven factors, but our knowledge of the processes underlying disease initiation and pathology remains incomplete. Recent studies point to an important role of T cells in supporting auto-antibody production; yet the involvement of the myeloid compartment remains unexplored. Clinical management of pemphigus is beginning to move away from broad-spectrum immunosuppression and towards B-cell-targeted therapies, which reduce many patients' symptoms but can have significant side effects. Here, we review the latest developments in our understanding of the predisposing factors/conditions of pemphigus, the underlying pathogenic mechanisms, and new and emerging therapies to treat these devastating diseases., Competing Interests: BJ receives research funding from Evonik. PM is a consultant for Servier. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Lim, Bohelay, Hanakawa, Musette and Janela.)

Perhaps surprisingly, the manifestations and management of patients with pemphigus vulgaris and oral lesions have been detailed only infrequently. The present study has examined the clinical features, diagnosis and management of a cohort of 55 patients, including three adolescents, with pemphigus vulgaris predominantly affecting the oral mucosa. There was about a 6-month delay from the onset of symptoms until presentation for diagnosis, longer in men than in women. Patients typically had multiple lesions affecting mainly the buccal and/or palatal mucosae, and over half the patients had lesions affecting non-oral mucosal sites. Nearly one-quarter (24%) had cutaneous involvement. Most patients were otherwise healthy with no other autoimmune disorders. Classical histopathological features of pemphigus vulgaris were present in all patients, as well as IgG intraepithelial deposits in all patients tested and circulating epithelial antibodies in most. Thirty-two patients were treated in the clinic, four responding to topical immunosuppressive therapy, the remainder needing and responding, at least in part, to systemic immunosuppression. Systemic corticosteroids often with adjunctive immunosuppressives, particularly azathioprine, were required in 87% of patients. In 18% of the patients, the disease resolved in 3 months, but 76% had recalcitrant disease. Adverse effects were seen in 78%, and two patients died, at least one as a consequence of immunosuppressive therapy. It is concluded that pemphigus vulgaris affecting the oral mucosa is still diagnosed only after considerable delay because patients, especially men, present late; it has a chronic course; it is often associated with lesions in other mucosae and/or skin; it can be resistant to currently available therapies; and immunosuppressive therapy frequently produces adverse effects, occasionally lethal. [ABSTRACT FROM AUTHOR]

AbstractBackground Tetracycline is an antibiotic which has been proven beneficial as an immunomodulating drug in the treatment of autoimmune bullous diseases. Methods Thirteen hospitalized patients with pempigus vulgaris received tetracycline 2 g/day for 1 month, then 1 g/day for the following 4 weeks. Prednisone 0.5–1 mg/kg/day was then gradually reduced. The control group consisted of seven patients with pemphigus who had received prednisone and azathioprine. Results A total of 13 patients in the study group achieved cessation of new blister formation within 5.4 days, compared to 23.71 days (p < 0.0001). The average initial dose of prednisone was 76.53 mg/day for the study group and 118.57 mg/day in the control group (p < 0.014). The average number of days before reduction of the prednisone dosage could begin was 16.53 days for the study group compared to 31.28 days in the control (p < 0.049). Total hospitalization time was also significantly lower: 34.07 days (p < 0.001). The clinical response was not linked to the location of lesions nor to severity of disease. Conclusions Tetracycline was shown to be effective as an adjuvant therapy for pemphigus with low toxicity and safety. [ABSTRACT FROM AUTHOR]

Pemphigus vulgaris (PV) is a rare but potentially fatal autoimmune bullous disorder which frequently affects the oral mucosa. Although the essential cause of PV is unclear, its onset has occasionally been associated with drug therapy, in particular penicillamine. The patient described in this paper was a 78-year-old diabetic man who developed oral lesions of PV following institution of glibenclamide therapy. [ABSTRACT FROM AUTHOR]

The freeze-fracture technique was used to study acantholytic cells of untreated pemphigus patients and the cells of histocytosis X. On the membranes of acanthocytosis cells, the particles are randomly distributed and lack the typical desmosome organization, while tight and gap junctions are still found. The study of the cells of histocytosis X provided a better understanding of the structure of the Langerhans granule. The paracrystalline organization of the inner face of the disc-shaped portion does not seem to involve the membranes limiting the granule, but rather to be confined to a periodically arranged, possibly proteic substance contained between them. [ABSTRACT FROM AUTHOR]

At present, initial high-dose prednisone i the treatment of choice for patients with pemphigus and bullous pemphigoid. To reduce the risks associated with long-term corticosteroid treatment, other immunosuppressants are often given as steroid-sparing agents. Occasionally, the dose of steroids cannot be reduced. In this study, we report six patients with pemphigus vulgaris. pemphigus foliaceus and hullous pemphigoid, in whom the daily corticosteroid dose could only be tapered to acceptable, effective, maintenance levels following treatment with high-dose intravenous gammaglobulin. [ABSTRACT FROM AUTHOR]

Cyclophosphamide was ineffective in controlling acute disease activity in nine of eleven pemphigus patients, but when given during the remission phase even 50 mg cyclophosphamide per day was sufficient to prevent subsequent relapses in all the patients for quite long periods of follow-up. This small dose of cyclophosphamide could be given to outpatients and there were no side effects. [ABSTRACT FROM AUTHOR]

Background. Patients with pemphigus vulgaris may occasionally present the highly active, widespread form of the disease, and/or maybe resistant to conventional oral steroid therapy, and/or manifest significant side effects from the prolonged use of high oral prednisone dosage. Our purpose was to evaluate the efficacy of steroid "pulse" therapy (PT) in these patients Methods. Eight patients were given alternate-day, one hour, infusions of 8, 9, or 10 mg/kg methylprednisolone Na succinate. Oral prednisone and a second immunosuppressive agent were simultaneously administered; these were rapidly decreased when control of the disease was achieved. Results. All patients initially responded well to therapy. The disease recurred in four patients after 3, 4, 9, and 16 months of remission, respectively. Three of these patients were treated again with PT and went into remission. None of the patients who received cyclophospharnide had a recurrence. One patient died of cardiac arrest, 12 days after PT. On a follow-up of 59 ± 25 months since the last relapse, patients continue in remission. Conclusions. Pulse therapy is recommended as an adjunct to the total management plan of severely affected patients with pemphigus vulgaris. The addition of cyclophosphamide may prevent the disease from recurring. Alternate-day small- quantity bolus infusions over 16-20 days, may be equally effective as the administration of 15 mg/kg/daily for 3-5 days. The risk of cardiac arrest exists even with this, less aggressive form of PT therapy. A medical history of supraventricular arrhythmias may be considered a risk factor. [ABSTRACT FROM AUTHOR]

Six patients with proven pemphigus vulgaris have been treated with gold. Serious toxicity did not occur but "pseudotoxicity" was common. During the initial phase of treatment two patients developed a rash that cleared quickly. They both had a long history of mild minimal disease and refused further treatment. Although the natural history of pemphigus vulgaris has not definitely been documented it would seem that gold has beneficially influenced disease activity in these patients. [ABSTRACT FROM AUTHOR]

Seven children had pemphigus: six had pemphigus vulgaris and one pemphigus foliaceus. Four who had pemphigus vulgaris were administered dexamethasone pulses. Follow-up from six months to one year was uneventful; the disease was under control and there were no complications due to therapy. Our observations suggest that the clinical features, course, and principles of therapy for pemphigus in children are essentially the same as those in adults. [ABSTRACT FROM AUTHOR]

A male infant with skin lesions was born to a 28-year-old mother who was under treatment for pemphigus vulgaris (PV), diagnosed eight years earlier. Circulating IgG class pemphigus antibody was found in the infant's blood, and deposition of IgG in the intercellular spaces of the epidermis was seen. The infant's lesions resolved within three weeks, and pemphigus antibody titer became negative by seven weeks. The pathogenetic role of PV antibodies and the risk for a fetus of a mother suffering from PV are discussed. [ABSTRACT FROM AUTHOR]

Background: Although gold has been reported to be useful in treating pemphigus vulgaris, its use has waned in recent years because of concerns regarding efficacy and toxicity. Objective: To review 26 patients with pemphigus who were treated with intramuscular gold over a 10-year period. Results: Gold was effective in 62% of patients as a primary treatment for pemphigus or as a steroid-sparing agent. An average of 3 months of therapy was required before the daily prednisone dosage could be halved. Four patients were free of disease and stopped receiving all therapy at the conclusion of the study. Toxic effects due to gold therapy developed in 42% of patients and all adverse effects resolved with its cessation. Conclusions: While toxic effects limit the use of gold in many patients with pemphigus, it may be effective in treating a large percentage of patients who otherwise are unable to reduce their steroid requirement. Because of its delayed onset of action, patients treated with gold usually require systemic steroids when therapy is initiated. Controlled, prospective trials are needed to further evaluate the efficacy of gold and its potential steroid-sparing effects. [ABSTRACT FROM AUTHOR]

Hailey-Hailey disease (HHD) is a chronic familial bullous disease characterized by recurrent blisters and erosions typically at friction-prone areas of the body accompanied by acantholysis upon histologic examination. There are a number of therapies used in the management of HHD. Its symptoms have been effectively treated with antimicrobial therapies, corticosteroids and other agents such as cyclosporine and prednisone. However, such treatments are not always effective. Therefore, there is a need for new treatments for the management of HHD. In this report, a patient with long-standing HHD responsive only to high levels of prednisone is described. After the successful tapering and cessation of oral prednisone the patient began a new combination therapy of complementary doses of oral alitretinoin, and narrowband UVB therapy, which yielded a favorable response within 2-3 weeks. After 6 weeks, a mono-therapy of daily (30 mg) oral alitretinoin was sufficient to maintain successful near-complete remission of the disease. [ABSTRACT FROM AUTHOR]

A patient with an 11-year history of a generalized blistering eruption is described. The eruption was completely controlled within i week of taking sulphapyridine and recurred within 4 days of withdrawal of this drug. On account of these observations a diagnosis of dermatitis herpetiformis was made. Histological, immunological (and small intestinal) studies now show that this patient had pemphigus. [ABSTRACT FROM AUTHOR]

The article discusses several aspects of the systemic treatment with steroid hormones. Topics discussed include the cases in which steroids are life-saving, such as pemphigus, sytemic L.E., erythrodermas, urticaria, angio-neurotic edema, and erythema multiforme. In addition, reference is made to the protocol in dealing with atopic dermatitis, and cases of eczematous.

The article presents several case studies of patients suffering from various dermatological disorders including Acrodermis Chronica Atrophicans, Ocular Pemphigus, and Parapsoriasis. Treatments administered include Cortisone, X-ray therapy, and aureomycin, and the response of each patient to the treatment they received is described.

Results of treatment of eight Patients with South American pemphigus foliaceus with methotrexate are reported. All the patients continued to receive corticosteroids during the methotrexate therapy. In five patients there was no clinical improvement; in two a slight response was noted. One patient had a good response before his death from bronchopneumonia. No favorable alterations in the titers of antiepithelium antibodies and antistreptolysin "0" were noted. Those results suggest that this drug is not effective for pemphigus foliaceus. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

We report the case of successful treatment of a 79-year-old male patient with recurrent pemphigus foliaceus with pimecrolimus cream 1% once daily for 40 days. The patient initially presented with localized lesions on the scalp and nose area and was treated with systemic corticosteroids. At his fourth relapse within a period of 16 months, he refused any systemic treatment. Pimecrolimus cream was suggested to him as an alternative option. [ABSTRACT FROM AUTHOR]

We report a case of neutropenic ulceration in a 42-year-old woman receiving azathioprine for pemphigus vulgaris. She developed multiple indolent ulcers involving the nose, neck, and back, after about 6-8 weeks following commencement of azathioprine 50 mg daily. The ulcers were large, disfiguring, dry, and with basal necrotic slough. They were painless and did not discharge pus. The absolute neutrophil count was severely depressed initially, but normalized following azathioprine withdrawal. Swab culture revealed colonization with Klebsiella pneumoniae and the ulcers healed with local debridement, treatment with imipenem, and topical application of mupirocin. However, nasal disfigurement persisted. Neutropenic ulceration is known to be associated with azathioprine therapy but we report this case because of the unusual presentation-indolent cutaneous ulcers. Early recognition of the problem and drug withdrawal can prevent complications like disfigurement. [ABSTRACT FROM AUTHOR]

D-penicillamine is one of the disease-modifying anti-rheumatic drugs (DMARDs). Drug-induced pemphigus is not frequently associated with D-penicillamine, and to date, the number of reported cases is about a hundred. Most reports of D-penicillamine-induced pemphigus vulgaris are in patients with rheumatoid arthritis. It has rarely been reported in patients not taking D-penicillamine. We report a case of pemphigus vulgaris in a 48-year-old female patient with rheumatoid arthritis, not taking penicillamine. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented regarding the efficacy of rituximab in patients with refractory bullous pemphigoid (BP) and mucous membrane pemphigoid (MMP).

Autoimmune blistering skin diseases, including pemphigus vulgaris, rarely involve the esophagus. We report a case of exfoliative esophagitis with pemphigus vulgaris. A sloughing esophageal cast observed by endoscopy was dissected esophageal squamous epithelium in all layers. Our case is the fifth case of pemphigus vulgaris associated with esophageal cast formation recorded in the medical literature. Prednisolone was administered, and both the pemphigus vulgaris and exfoliative esophagitis improved. Upon findings of exfoliative esophagitis by endoscopic examination, we should consider the coexistence of blistering skin diseases, including pemphigus vulgaris. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 52-year-old woman with lesion on her neck and had developed pemphigus foliaceus (PF) three years previously. A small papule and bowenoid solar keratosis were noted on physical examination and histological examination respectively. The patient has experienced a relapse of PF within one week of imiquimod treatment. Prednisone and topical mometasone cream treatments were started which resolved the bullae and removed the basal cell carcinoma (BCC).

Abstract: Pemphigus foliaceus is a rare autoimmune blistering disease comprising two major types: endemic and sporadic. The endemic form, also known as fogo selvagem, affects children and young adults in rural Brazil while the sporadic form generally affects the middle-aged and the elderly. Here we report a sporadic form of pemphigus foliaceus in a child in which the skin eruption responded to flucloxacillin. Upon discontinuation of flucloxacillin, lesions reappeared and then cleared upon resuming the antibiotic. Dapsone was thereafter administered with good control. [Copyright &y& Elsevier]

Pemphigus vulgaris is a rare, life-threatening autoimmune disease. Mycophenolate mofetil is a potent immunosuppressant medication approved by the Food and Drug Administration to be used after solid organ transplantation and to treat pemphigus vulgaris. Mycophenolate mofetil has not become the “wonder drug” that had been anticipated based on initial clinical reports. Studies like that reported by Beissert et al. in this issue are essential to improve dermatologic care. [ABSTRACT FROM AUTHOR]

Abstract: The autoimmune bullous skin disorder pemphigus is characterized by the presence of autoantibodies that target distinct adhesion molecules of the epidermis. The autoantibodies result in a loss of the epidermal adhesive properties, which in turn leads to the appearance of blisters and erosions on skin and mucosal surfaces. In the majority of cases, disease activity can be sufficiently controlled by systemic corticosteroids in combination with immunsuppressants, but pemphigus often is recalcitrant to conventional immunosuppressive therapy. Clinical remission can be obtained only in a minority of patients. In the past, only cyclophosphamide and high-dose intravenous immunoglobulin (IVIG) were available as second-line therapies. Meanwhile, immunoadsorption and monoclonal anti-CD 20 antibody rituximab have been established as further therapeutic options. [Copyright &y& Elsevier]

The article presents a case study on patients with pemphigus vulgaris (PV) that underwent genetic analysis of desmoglein 3 (DSG3) sequence variants in India and Great Britain. Researchers found that PV is caused by autoantibodies directed against epithelial adhesion protein desmoglein 3. Moreover, DSG3 coding plays a vital role in PV susceptibility.

A letter to the editor is presented about the dexamethasone-cyclophosphamide pulse (DCP) therapy for the pemphigus group of disorders.

Pemphigus is an autoimmune blistering disease affecting skin and mucous membranes which threatens the life. In our country it is the most common disease in this group. In our region, among the pemphigus cases, the most common variant is pemphigus vulgaris. Pemphigus vulgaris consists of the 80% of pemphigus cases and it occurs 10 times more than pemphigus foliaceus. Treatment of pemphigus is accepted as a miracle in clinical medicine. The disease, which had an almost always fatal outcome, had been turned to the disease which long-term remissions or "cure" can be achievable. However, in the past the cause of the death was the disease itself, nowadays, with decreasing frequency, all of the mortalities is due to the treatment side effects. In treatment of pemphigus which drug to use and when to use it has varieties according to the intended effect. Corticosteroids are the main treatment; besides IVIg, plasmapheresis or pulse steroid prefers to control the disease rapidly. Mainly immunosuppressive agents (azathioprine, methotrexate, cyclophosphamide, cyclosporine, and mycophenolate) besides gold, dapsone, antibiotics or rituximab are using for late-term effect and to reduce the corticosteroid requirement. [ABSTRACT FROM AUTHOR]

This article presents an editorial commenting on a study in this issue on the use of rituximab for the treatment of pemphigus. Pemphigus is an autoimmune disease which seems to respond well to immune globulins and B-cell-targeted humanized monoclonal antibodies. The author suggests that physicians use rituximab for only those patients who have the disease that is not responsive to therapy with corticosteroids and immunosuppressive agents because of the risk of serious short-term complications.

A letter to the editor on the treatment of benign familial pemphigus is presented.

The article presents several abstracts on clinicopathological studies. Included are "Lichen Sclerosus and Eosinophilic Fasciitis as Manifestations of Chronic Graft-Versus-Host Disease: Expanding the Sclerodermoid Spectrum," by J. V. Schaffer, J. M. McNiff, S. Seropian and others, "Serum Interleukin-8 as a serologic Marker of Activity in Behcet's Disease," by G. Gür-Toy, N. Lenk, B. Yalcin and others and "Anti-CD20 Monoclonal Antibody (Rituximab) in the Treatment of Pemphigus," by M. J. Arin, A. Engert, T. Krieg and others. "

We describe a rare case of pemphigus foliaceus associated with familial myasthenia gravis (MG). A 35-year-old woman developed MG during oral corticosteroid treatment for pemphigus foliaceus. She had been operated on for a thyroid gland tumour that was confirmed histopathologically to be papillary carcinoma without metastasis. At the time of treatment, her mother had had MG for 30 years and undergone thymectomy 22 years ago. A specific ELISA technique showed that antidesmoglein 1 antibody was present in the daughter. There are many reports of multiple diseases such as pemphigus, thymoma, malignancy, and other autoimmune diseases associated with MG. However, familial MG following pemphigus foliaceus has not been reported previously. [ABSTRACT FROM AUTHOR]