Use of Complementary and Alternative Medicine in Patients with Autoimmune Bullous Dermatoses: A Cohort Study Analysis of a Rare Disease Group.

INTRODUCTION: Autoimmune bullous diseases (AIBD) are a heterogeneous group of rare autoantibody-mediated blistering dermatoses of the skin and/or mucous membranes. Their incidence is around 20 new cases per million inhabitants per year in Germany. Patients with chronic, oncological, or rare diseases often urge for a holistic therapeutic approach that includes complementary and alternative medicine (CAM). So far, only few contradictory reports on CAM in pemphigoid or pemphigus disease exist. The purpose of this study was to determine the frequency, motives, and satisfaction with the use of alternative treatments in patients with AIBD and to provide a basis for further investigation. METHODS: We used a structured online questionnaire, consisting of 20 questions to survey patients with AIBD and their relatives. The German pemphigus and pemphigoid self-help groups were responsible for distributing anonymized questionnaires. In total, we recovered 97 questionnaires, 63 of which met full inclusion criteria (24 males and 39 females). RESULTS: Of the included participants, more than half had a currently active disease. Of all patients, 58.7% stated that they had used CAM at least once. Women were more likely to use CAM, whereas age and education showed no association to CAM use. The main motives for using CAM were "doing something for oneself" and "opportunity to contribute to treatment" (38.1% each). The internet (23.8%) was the most common source of information, and vitamins were the most frequently used therapy (49.2%). CONCLUSION: Our results provide new insights into the demand for CAM within this rare disease patient group. Physicians should be aware of these methods to meet patient needs but also be able to identify potential barriers such as risks and interactions.

Biological therapy of autoimmune blistering diseases.

INTRODUCTION: Autoimmune blistering skin diseases are a group of disorders subdivided according to the location of blister formation: intraepidermal blistering in the pemphigus group and subepidermal in the pemphigoid group. These conditions are clinically heterogeneous and are treated with systemic corticosteroids and/or other forms of immunosuppression on the basis of clinical subtype and disease severity. These approaches may not be effective for the induction and maintenance of clinical response or need to be stopped because of intolerable side effects. AREAS COVERED: Biological therapies can represent a valid alternative strategy in various autoimmune blistering disorders and this review article will address this issue with a special focus on pemphigus vulgaris and bullous pemphigoid. These biological approaches are designed to target B cells, autoantibodies, complement proteins, and several cytokines. EXPERT OPINION: Innovative strategies for the treatment of autoimmune blistering conditions primarily depend on the use of drugs with a high degree of specificity targeting crucial steps in the immunopathology of these disorders. Novel biological agents offer treatment alternatives to patients with autoimmune blistering conditions by targeting B cells, pathogenic autoantibodies, complement and cytokines.

Penfigoide buloso nodular tratado exitosamente con fototerapia UVB banda angosta / Pemphigoid nodularis successfully treated with narrow band UVB phototherapy

El Penfigoide nodular es una variante clínica poco frecuente de penfigoide buloso. Corresponde a una dermatosis ampollar subepidérmica, crónica, autoinmune, caracterizada por auto anticuerpos contra antígenos específicos de hemidesmosomas en la unión dermo-epidérmica. Su incidencia es desconocida. La etiopatogenia aún no es entendida del todo. Se presenta clínicamente como una superposición de características de pénfigo buloso y prurigo nodular. El diagnóstico se basa en hallazgos clínicos e inmunopatológicos. La histopatología con inmunofluorescencia directa es el gold standard para el diagnóstico. El manejo es difícil, tiene mala respuesta a corticoides potentes locales, siendo necesario el uso de corticoides sistémicos y diferentes inmunosupresores solos o combinados junto a antihistamínicos para el manejo de prurito intenso. Se presenta un caso de pénfigo nodular, donde destaca su buena respuesta a terapia combinada con metotrexato y luz UVB de banda angosta.

Pemphigoid Nodularis is a rare clinical variant of bullous pemphigoid. It is considered an autoimmune, chronic, subepidermal blistering dermatosis, characterized by antibodies against hemidesmosome-specific antigens at the dermo-epidermal junction. Its incidence is unknown and its etiopathogenetic not fully understood. Clinically, it presents with overlapping features of bullous pemphigoid and prurigo nodularis. The diagnosis is based on clinical and immunopathological findings, being the histopathological study with immunofluorescence the gold standard. The management is difficult; since it has a poor response to local potent corticosteroids, requiring the use of systemic corticosteroids and different immunosuppressants alone or combined with antihistamines for the intense pruritus. We present a case of nodularis pemphigoid, highlighting the good response to the combination of methotrexate and phototherapy with narrow band UVB.

Management of bullous pemphigoid: the European Dermatology Forum consensus in collaboration with the European Academy of Dermatology and Venereology.

Bullous pemphigoid is the most common autoimmune subepidermal blistering disease of the skin and mucous membranes. This disease typically affects the elderly and presents with itch and localized or generalized bullous lesions. In up to 20% of affected patients, bullae may be completely absent, and only excoriations, prurigo-like lesions, eczematous lesions, urticated lesions and/or infiltrated plaques are observed. The disease is significantly associated with neurological disorders. The morbidity of bullous pemphigoid and its impact on quality of life are significant. So far, a limited number of national treatment guidelines have been proposed, but no common European consensus has emerged. Our consensus for the treatment of bullous pemphigoid has been developed under the guidance of the European Dermatology Forum in collaboration with the European Academy of Dermatology and Venereology. It summarizes evidence-based and expert-based recommendations.

[Bullous pemphigoid: a review]. / Pemphigoïde bulleuse: revue de la littérature.

BACKGROUND: Bullous pemphigoid (BP) is the most common auto-immune bullous disorder. Its treatment is difficult due to high age and comorbidities of affected patients. OBJECTIVES: To assess the effects of treatments for BP. METHODS: Randomized therapeutic trials (RCTs) were identified using an automatic search on Pubmed et Embase until March 2009. Large retrospective series with homogeneous therapeutic management were also selected and analyzed. RESULTS: Forty-four articles were selected and analyzed, which included nine RCTs with a total of 1007 participants (653 patients were included in two trials). Two RCTs comparing different modalities of systemic corticosteroid therapy failed to show differences in measure of disease control. The addition of plasma exchanges (one RCT) or azathioprine (one RCT) allowed to halve the amount of prednisone required for disease control. A further 3-arms RCT compared plasma exchange or azathioprine plus prednisone, but failed to show significant differences for disease control or mortality of BP. One study compared tetracycline plus nicotinamide with prednisolone, no significant difference for disease response was evidenced. A large controlled clinical trial demonstrated that high doses of very potent topical corticosteroids increased initial disease control and 1-year survival of patients with extensive BP, as compared with oral prednisone. Another RCT compared two regimens of potent topical corticosteroids and a non-inferior rate of BP control was obtained with the mild regimen. Finally, a study comparing two immunosuppressant drugs (azathioprine, mycophenolate mofetil) in addition to prednisone failed to show any difference for disease control, recurrence rate or the cumulated doses of prednisone. CONCLUSIONS: Ultrapotent topical corticosteroids (clobetasol propionate; 20 to 40g/day) are effective treatments for BP with fewer systemic side-effects than oral high-dose corticosteroids. Systemic corticosteroids are effective but doses greater than 0.5mg/kg per day are associated with severe side-effects, including decreased survival. The effectiveness of the addition of plasma exchange or immunosuppressants (azathioprine, mycophenolate mofetil) to systemic corticosteroids has not been established. Combination treatment with tetracycline and nicotinamide needs further validation.

Interventions for bullous pemphigoid.

BACKGROUND: Bullous pemphigoid (BP) is the most common autoimmune blistering disease in the West. Oral steroids are the standard treatment.This is an update of the review published in 2005. OBJECTIVES: To assess treatments for bullous pemphigoid. SEARCH STRATEGY: In August 2010 we updated our searches of the Cochrane Skin Group Specialised Register, the Cochrane Central Register of Controlled Trials (Clinical Trials), MEDLINE, EMBASE, and the Ongoing Trials registers. SELECTION CRITERIA: Randomised controlled trials of treatments for participants with immunofluorescence-confirmed bullous pemphigoid. DATA COLLECTION AND ANALYSIS: At least two authors evaluated the studies for the inclusion criteria, and extracted data independently. MAIN RESULTS: We included 10 randomised controlled trials (with a total of 1049 participants) of moderate to high risk of bias. All studies involved different comparisons, none had a placebo group. In 1 trial plasma exchange plus prednisone gave significantly better disease control at 1 month (0.3 mg/kg: RR 18.78, 95% CI 1.20 to 293.70) than prednisone alone (1.0 mg/kg: RR 1.79, 95% CI 1.11 to 2.90), while another trial showed no difference in disease control at 6 months.No differences in disease control were seen for different doses or formulations of prednisolone (one trial each), for azathioprine plus prednisone compared with prednisone alone (one trial), for prednisolone plus azathioprine compared with prednisolone plus plasma exchange (one trial), for prednisolone plus mycophenolate mofetil or plus azathioprine (one trial), for tetracycline plus nicotinamide compared with prednisolone (one trial). Chinese traditional medicine plus prednisone was not effective in one trial.There were no significant differences in healing in a comparison of a standard regimen of topical steroids (clobetasol) with a milder regimen (RR 1.00, 95% 0.97 to 1.03) in one trial. In another trial, clobetasol showed significantly more disease control than oral prednisolone in people with extensive and moderate disease (RR 1.09, 95% CI 1.02 to 1.17), with significantly reduced mortality and adverse events (RR 1.06, 95% CI 1.00 to 1.12). AUTHORS' CONCLUSIONS: Very potent topical steroids are effective and safe treatments for BP, but their use in extensive disease may be limited by side-effects and practical factors. Milder regimens (using lower doses of steroids) are safe and effective in moderate BP. Starting doses of prednisolone greater than 0.75 mg/kg/day do not give additional benefit, lower doses may be adequate to control disease and reduce the incidence and severity of adverse reactions. The effectiveness of adding plasma exchange, azathioprine or mycophenolate mofetil to corticosteroids, and combination treatment with tetracycline and nicotinamide needs further investigation.

Penfigoide ampolloso relacionado con tratamiento con PUVA: aportación de dos nuevos casos / Bullous pemphigoid related to PUVA therapy: Two further cases

El penfigoide ampolloso es una enfermedad de etiología autoinmune caracterizada por el desarrollo de ampollas subepidérmicas, que afecta generalmente a personas de edad avanzada. A pesar de que el penfigoide ampolloso tiene un potencial carácter fotosensible, se ha descrito de forma excepcional el desarrollo de dicho proceso en el curso de tratamiento con PUVA, sobre todo en pacientes con psoriasis. La asociación de penfigoide ampolloso y psoriasis plantea dificultad a la hora de instaurar un tratamiento y consideramos una buena alternativa la utilización de metotrexate, con o sin corticoides asociados, en el manejo de dichos pacientes. Aportamos dos nuevos casos de penfigoide ampolloso relacionado con tratamiento con PUVA en pacientes con psoriasis

Bullous pemphigoid is an autoimmune disease that generally affects elderly people and is characterised by the development of subepidermal blistering. Although bullous pemphigoid is potentially photosensitive, its occurrence during the treatment course with PUVA, especially in patients with psoriasis, has exceptionally been described. The association of bullous pemphigoid and psoriasis gives rise to difficulties when initiating treatment and we consider that the use of methotrexate, with or without associated corticoids, is a good alternative in the management of such patients. We report two further cases of bullous pemphigoid related to PUVA therapy in patients with psoriasis

Childhood bullous pemphigoid: report of a case with life-threatening course during homeopathy treatment.

Bullous pemphigoid (BP) is an autoimmune blistering disorder that may very rarely occur in childhood. We describe a 9-month-old child who developed bullous pemphigoid while she was being treated for presumptive atopic eczema with a homeopathic regimen comprising sulfur, mercury, cantharides, and Rhus (Toxicodendron). She had generalized bullae and a progressive worsening of her general condition with asthenia, dehydration, malnutrition. While the role of homeopathy in triggering the disease remains unclear, our observation attests to the potential life-threatening course of childhood BP in instances where appropriate treatment is withheld.

Intravenous immunoglobulin therapy for patients with bullous pemphigoid unresponsive to conventional immunosuppressive treatment.

BACKGROUND: Up to 24% of patients with bullous pemphigoid (BP) do not respond to conventional therapy consisting of oral prednisone alone or combined with corticosteroid-sparing immunosuppressive agents (ISAs). They cannot sustain a prolonged clinical remission and continue to have relapses. OBJECTIVE: Fifteen patients with recurrent BP who had experienced several significant side effects resulting from conventional therapy were treated with intravenous immunoglobulin (IVIg) therapy. METHODS: A preliminary dose-finding study tested 7 additional patients to ascertain the optimal IVIg dose of 2 gm/kg per cycle. Objective parameters to determine clinical outcomes were recorded before and after IVIg therapy: doses of prednisone and ISAs, their duration, side effects, clinical course, frequency of relapses and recurrence, response to therapy, number of hospitalizations, total days hospitalized, and quality of life. RESULTS: While receiving IVIg as monotherapy, all study subjects achieved a sustained clinical remission. A statistically significant difference was noted in all the variables studied before and after IVIg therapy. IVIg had a corticosteroid-sparing effect and improved quality of life and did not produce any serious side effects. CONCLUSION: IVIg appears to be an effective alternative in treating patients with severe BP whose disease is nonresponsive to conventional therapy. IVIg may be particularly useful, if treatment is begun early, in patients who are at risk of experiencing serious or potentially fatal side effects from conventional immunosuppressive therapy. After clinical control is achieved, IVIg therapy should be gradually withdrawn and not abruptly discontinued.

The alterations of keratinocyte surface and basement membrane markers by treatment with 8-methoxypsoralen plus long-wave ultraviolet light.

The alterations of keratinocyte surface and basement membrane markers by treatment with 8-methoxypsoralen (8-MOP) plus long-wave ultraviolet (UVA) light (PUVA) were examined in guinea pig skin using immunofluorescence (IF) microscopy. Following a single PUVA treatment (UVA doses: 1.8 - 7.2 J/cm2), reactivity with pemphigus and pemphigoid sera, rabbit anti-guinea pig epidermal cell serum, and fluorescein-conjugated concanavalin A was decreased in intensity in a dose-dependent fashion. Fluorescence became distinctly less pronounced by day 2 postirradiation and recovered between days 6-10. IF with rabbit antiglomerular basement membrane antibodies, which cross-reacted with the basement membrane of the skin, apparently was unaltered within this energy dose range. 8-MOP or UVA alone did not change the reactivity with any reagent. These results suggest that PUVA treatment affects keratinocyte membrane markers and pemphigoid antigens but not some of the basement membrane antigens.