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Guidelines for the management of bullous pemphigoid
글 올린 시간 January 12, 2010 글 쓴이 webadmin
짹 짹짹	These guidelines have been prepared for dermatologists on behalf of the British Association of Dermatologists. They present evidence-based guidance for treatment, with identification of the strength of evidence available at the time of preparation of the guidelines and a brief overview of epidemiological aspects, diagnosis and investigation. The guidelines reflect data available from Medline, Embase, the Cochrane library, literature searches and the experience of the authors of managing patients with bullous pemphigoid in special and general clinics for over 10 years. 그러나, caution should be exercised in interpreting the data obtained from the literature because only six randomized controlled trials are available involving small groups of patients.
BP is a non-scarring blistering disease, typically with a flexural distribution of skin lesions. 그러나, the disease may be generalized or may be localized to one site. Mucous membranes are involved in about 50% off 환자, with the oral mucosa most frequently affected. Tense blisters arise on either erythematous or normalappearing skin. Oral lesions consist of small blisters or erosions and are found mainly on the palatal mucosa. The blister formation may be preceded by an urticarial or eczematous rash. The degree of itch varies from none to intense and may precede the appearance of blisters by weeks, months or occasionally years.
The class of immunoglobulin bound to the basement membrane zone on direct IF distinguishes linear IgA disease (LAD) (only IgA on direct IF) from BP. Indirect IF performed on salt-split skin will differentiate BP from epidermolysis bullosa acquisita (EBA) and from a subgroup of cicatricial pemphigoid (CP). The antibodies are detected at the roof of the artificial blister in BP and at the base in laminin 5-CP and in EBA. 그러나, this is not relevant to most clinical practice, as both CP and EBA are far rarer diseases and none of the published controlled clinical trials in BP has used this method to classify patients.
The efficacy of systemic corticosteroid treatment in BP was demonstrated in uncontrolled clinical studies and is well established in clinical experience.13–15 그러나, few studies are directly comparable because patients differ in severity of disease and there are also differences between treatment regimens, therefore optimum dosage schedules remain a subject for debate. The corticosteroids most commonly used are prednisolone and prednisone, and dosages relate to these drugs unless otherwise stated. Typical recommendations for widespread disease are for a starting daily dose of about 1 mg kg)1 continued until cessation of new blister formation, then gradually decreased according to clinical course.11,16,17 그러나, many studies do not closely relate corticosteroid dose to body weight, and tend to use a uniform starting dose, usually ranging between 40 and 80 mg daily, typically 60 mg daily.14 More recently, lower starting doses of 20–40 mg daily have been recommended.18
There is some evidence, one small RCT12 , small uncontrolled trials, and case reports that antibiotics and nicotinamide (niacinamide) should be considered as the first line of treatment for both localized and mild to moderate disease (Strength of recommendation B, Quality of evidence II-ii ⁄ iii). There are 38 reports (183 환자) of BP treated with tetracycline or erythromycin, often in combination with nicotinamide and sometimes with topical or even oral corticosteroids. Occasional blister formation was accepted in most reports. There are only two case series involving 11 and 15 환자, and many case reports, of the beneficial effect of erythromycin in children and adults.29–31 Erythromycin should be considered for treatment, particularly in children (adult dose 1000–3000 mg daily), and perhaps in combination with topical corticosteroids. A beneficial effect may be seen within 1–3 weeks after commencing treatment (Strength of recommendation B, Quality of evidence II-iii).
After systemic corticosteroids, azathioprine in doses of up to 2Æ5 mg kg)1 daily is the most commonly used drug in BP. It is mostly employed as an adjunct to systemic corticosteroids for its presumptive steroidsparing effect. 그러나, the efficacy of azathioprine as a steroid-sparing agent in BP has been addressed in only two RCTs, with conflicting results (Table 1). One RCT reported a 45% reduction in cumulative prednisolone dosage over a 3-year period.9 Conversely, a larger RCT found no difference in remission rates at 6 months in patients treated with corticosteroids only compared with those receiving combination treatment with prednisolone and azathioprine11 (Quality of evidence IV).
Experience with cyclosporin is limited to five individual case reports and a small series of seven 환자. The evidence for benefit is conflicting, even with relatively high dosage, > 6 mg mg kg)
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