bullos pemphigoid

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    JOURNAL READING

    By : Vera Septia Nalurita (2008730129)

    Preceptor : dr. Bowo Wahyudi, SpKK

    RSUD KOTA BANJAR

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    Background

    Bullous pemphigus is a chronicdisease characterized by tense bullae

    walled, often accompanied by

    erythema Bullous pemphigus blistering skin

    disease most common in adults

    Parents have a low tolerance for oralcorticosteroid regimen

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    Purpose

    Is topical corticosteroid therapyeffective for both moderate and severe

    bullous pemphigoid, and for better oral

    corticosteroid therapy for extensivedisease?

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    Method

    A total of 341 patients with bullouspemphigoid were enrolled in arandomized, multicenter trial andstratified according to the severity of their

    disease (moderate or extensive). Patients were randomly assigned to

    receive either topical clobetasolpropionate cream (40 g per day) or oral

    prednisone (0.5 mg per kilogram of bodyweight per day for those with moderatedisease and 1 mg per kilogram per dayfor those with extensive disease).

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    Inclusion criteria

    patients with newly diagnosed bullous pemphigoid

    were eligible for entry if the following criteria weremet :

    Clinical features suggestive of bullous pemphigoid

    subepidermal blister on skin biopsy;

    and linear deposits of IgG and C3 along thebasement-membrane zone

    Exclusion criteria

    Exclusion criteria were predominant or exclusivemucosal involvement

    Treatment with oral or topical corticosteroids,dapsone, or immunosuppressive drugs during the

    previous six months.

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    Study Design

    This randomized, nonblinded studycompared two parallel groups of patients

    treated with topical or oral corticosteroids.

    Randomization was stratified according tothe clinical center and the severity of

    disease, which was determined on the

    basis of the mean number of new bullae

    that had appeared daily during the three

    previous days

    moderate disease was defined by 10 new

    bullae daily

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    The initial dose was maintained for 15 days

    after control of the disease had beenattained. Thereafter, the dose was reducedby 15 percent every 3 weeks. Treatment wasstopped after 12 months

    patients who were assigned to receive topicalcorticosteroids received a daily dose of 40 gof clobetasol propionate that was appliedtwice daily on the entire surface of the bodyuntil 15 days after control of the disease had

    been attained. The doses were then gradually reduced to

    20 g daily for one month, 10 g daily for twomonths, 10 g every other day for four months,

    and finally 10 g twice a week for four months

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    Statical Analysis

    The primary end point was overallsurvival during the first year after theonset of bullous pemphigoid

    Secondary end points were control ofthe disease at day 21 :the absence of new bullae for three

    consecutive days

    no severe side effects occur during thefirst year

    cumulative hospital days (including theinitial hospitalization plus all

    rehospitalizations).

    R lt

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    Result

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    Among both patients with moderate bullous pemp higoid and patients with

    extensive bullous pemphigoid, no significant differences were observed between

    patients assigned to receive topical applications of clobetasol propionate cream

    and those assigned to receive oral prednisone

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    Result

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    The corresponding figures among the patients with extensive disease were

    44 of the 95 patients in the oral-prednisone group (46 percent; 95 percent

    confidence interval, 36 to 56 percent) after a mean interval of 210133 days

    and 34 of the 93 patients in the topical-corticosteroid group (37 percent; 95

    percent confidence interval, 27 to 46 percent) after a mean interval of187118 days

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    Result

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    only cases of diabetes requiring insulin were included in the analysis of

    severe (grade 3) or life-threatening (grade 4) adverse events.

    Data include both clinically apparent fractures and vertebral fractures visible

    on radiography

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    Result

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    Students t-test was used to compare the mean durations of

    hospitalization

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    Discussion

    Systemic corticosteroids have beenconsidered the mainstay of treatment

    for bullous pemphigoid for 40 years.

    The present study confirms the poorprognosis of patients with bullous

    pemphigoid who are treated with 1 mg

    of prednisone per kilogram per day,among whom the one-year mortality

    rate was 41 percent

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    The present study was designed totest the hypothesis that topical

    corticosteroids could be an effective

    alternative treatment for patients withbullous pemphigoid and would result

    in a decrease in the incidence of

    severe adverse events Our results clearly demonstrate the

    efficacy of clobetasol propionate

    cream

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    Topical treatments have lower toxicitythan the 1 mg of prednisone per

    kilogram per day

    Topical corticosteroids should beconsidered as a standard treatment

    for patients with extensive bullous

    pempigous

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    Conclusion

    Topical corticosteroid therapy is

    effective for both moderate and

    severe bullous pemphigoid andis superior to oral corticosteroid

    therapy for extensive disease

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