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Chapter 40: Bullous Pemphigoid

Heather Holahan; Lauren Crouse; Luis A. Diaz

BULLOUS PEMPHIGOID

Therapeutic Approach

Bullous pemphigoid is an autoimmune, subepidermal, mucocutaneous, blistering disease that typically, but not exclusively, affects the elderly population, aged 65 years and older. Involvement consists of urticarial plaques and tense, fluid-filled blisters which can be localized to one body area or generalized across several. First-line therapy often consists of topical corticosteroids for mild localized disease and/or systemic glucocorticoids combined with immunosuppressive medications, IVIg, or biologics for moderate to severe disease. Therapeutics are not without adverse events and should be guided by the severity of disease (erosions/bullae/urticaria/erythema on body areas), risk of adverse events, and relevant patient comorbidities. See Table 40-1.

Table 40-1Bullous Pemphigoid Treatment Table
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Table 40-1 Bullous Pemphigoid Treatment Table
MEDICATION NAME INDICATION MECHANISM OF ACTION DOSING ADVERSE EFFECTS SUGGESTED MONITORING LEVEL OF EVIDENCE (REF)
Topical Therapy
Clobetasol Propionate Cream BP Anti-Inflammatory 10-40 g/d Skin Atrophy/Bruising none IA1
Systemic Therapy
Oral glucocorticoids (prednisone; prednisolone) BP Immunomodulatory

0.3 mg/kg/d for mild and 0.5-1 mg/kg/d in moderate and severe with

Vitamin D and Ca supplementation

Weight gain

Hypertension

Diabetes mellitus

Decreased bone density

Adrenal gland suppression

Blood glucose

Blood pressure

Body weight

Urine (dipstick)

Ophthalmologic exam

Monthly then every 2-3 mo2

 1A1, 3-7
Methotrexate BP Anti-metabolite 5-15 mg weekly po or im

Myelosuppression

GI upset

Infection

Liver and Pulmonary fibrosis

Teratogenicity

Pregnancy test (initial)

CBC w/diff, LFTs, 1 week after first dose then biweekly after dose escalation then 3-4 mo thereafter.

BUN/sCr every 6 mo-1 y2

 III8
Mycophenolate Mofetil BP Purine synthesis inhibitor 1-2 g BID

GI upset

Liver dysfunction

Hyperglycemia

Infection

Pregnancy test (initial), baseline serum chemistry, hepatitis B/C, PPD.

CBC w/diff, BUN/sCr, LFT’s every 2-4 wk after dose increase2 then every 2-3 mo2

 IIB9,10
Azathioprine BP Purine synthesis inhibitor 1.5-3 mg/kg/d

GI upset

Myelosuppression

Liver dysfunction

Infection teratogenicity

TPMT level (initial)

Pregnancy test, baseline CBC w/diff, serum chemistry, urinalysis, then CBC w/diff, LFTs biweekly for 2 mo, then every 2-3 mo

 III9,11,12
Cyclophosphamide BP Inhibits protein synthesis via DNA/RNA crosslinking 50-100 mg/d

Myelosuppression

GI upset

Hemorrhagic cystitis

Bladder Ca teratogenesis

CBC w/diff, Urinalysis weekly for 2-3 mo, then biweekly for 2-3 mo, and then monthly.

Serum chemistry, monthly for 3-6 mo, then 3-4 mo2

 III13-15
Limited Use            
Dapsone BP Anti-inflammatory; Anti-microbial 100-300 mg daily

Peripheral Neuropathy

Hemolytic Anemia

Methemoglobinemia

Hepatitis

Agranulocytosis

Hypersensitivity

G6PD level

CBC w/diff, weekly for 4 wk, then biweekly for 2 mo, and then 3-4 mo

LFTs, renal function, urinalysis 3-4 mo2

 III16,17
Doxycycline BP Anti-inflammatory; anti-microbial 100 mg QD-BID

GI upset

Photosensitivity

Esophagitis

Contraindicated in pregnancy and <8 yo

 none IB18,19
Minocycline BP Anti-inflammatory; anti-microbial 100 mg QD-BID

Contraindicated in pregnancy and <8 yo

Headache

Hyperpigmentation

Pseudotumor cerebri

 none IB19,20...

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