Table 4.1-1. Summary of topical and systemic agents used for the treatment of bullous pemphigoid

Drug

Dosage

Adverse effects

Special considerations

Superpotent topical glucocorticoids (0.05% clobetasol propionate cream or ointment)

Topically on affected areas once daily or bid

– Skin atrophy

– Acne

– Purpura

– Hypertrichosis

– Hypopigmentation

Should be avoided if there is extensive BSA involvement (>10%) due to risk of systemic absorption. Should not be used continuously for >3 weeks

Systemic glucocorticoids (prednisone)

Prednisone 0.5-1 mg/kg/d PO until full resolution of itching and blisters, then tapered down slowly over a few months

– Hyperglycemia

– Hypertension

– Peripheral edema

– Osteoporosis

– Myopathy

– Cataracts

– Glaucoma

– Peptic ulcer disease

– Leukocytosis

– Neutrophilia

– Lymphopenia

– Insomnia

– Irritability

– Mood changes

Tetracycline antibiotics (tetracycline, minocycline, doxycycline)

Tetracycline 500 mg PO qid or minocycline 100 mg PO bid, or doxycycline 100 mg PO bid

– GI upset

– Photosensitivity

– Drug-induced lupus with minocycline

Can be given with or without nicotinamide (vitamin B3). Nicotinamide is an over-the-counter product and has been used at doses of 500-2000 mg/d due to a possible anti-inflammatory effect

Azathioprine

TPMT level must be checked prior to therapy initiation. The maximum doses are 2 mg/kg/d (TPMT >19 U), 1-1.5 mg/kg/d (TPMT, 13.7-19 U), and 0.5 mg/kg (TPMT, 5-13.7 U)

– GI upset

– Myelosuppression

– Immunosuppression

– Increased risk of infection

– Increased risk of cancers including nonmelanoma skin cancers

– Pancreatitis

Requires follow-up monitoring of CBC, liver and kidney function tests

MMP or MPA

1-3 g/d (MMP) or 360-1080 mg bid (MPA)

– GI upset

– Myelosuppression

– Immunosuppression

– Increased risk of infection

– Increased risk of cancers

– Sterile pyuria

– Dizziness

Requires follow-up monitoring of CBC, liver and kidney function tests

Methotrexate

7.5-20 mg/wk PO or SC

– Nausea, vomiting, and GI upset

– Fatigue

– Leukopenia

– Alopecia

– Mucositis

– Elevated liver enzymes

– Pneumonitis

Requires follow-up monitoring of CBC, liver and kidney function tests

Dapsone

G6PD levels should be checked prior to initiating dapsone. The dose ranges from 25 to 100 mg/d PO

– Hemolytic anemia

– Methemoglobinemia

– Agranulocytosis

– Hypersensitivity reaction

– Peripheral neuropathy

IVIG

2-3 g/kg every 4 weeks

– Transfusion reactions

– Transfusion-associated circulatory overload

– Anaphylaxis in IgA-deficient patients

– Very expensive

– Premedication with acetaminophen (INN paracetamol) and antihistamines may prevent transfusion reaction or reduce its severity

Rituximab

375 mg/m2/wk IV × 4 doses or 1 g IV on days 0 and 15

– Infusion reactions (fever, headache, nausea, and pruritus) within the initial 0.5-2 h of the first infusion

Premedication with acetaminophen (INN paracetamol) and antihistamines, usually with glucocorticoids PO, is commonly used to prevent infusion reactions

BSA, body surface area; CBC, complete blood count; GI, gastrointestinal; G6PD, glucose-6-phosphate dehydrogenase; INN, international nonproprietary name; IVIG, intravenous immunoglobulin; MMP, mycophenolate mofetil; MPA, mycophenolic acid; PO, oral; SC, subcutaneous; TPMT, thiopurine methyltransferase.