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

Systemic glucocorticoids

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

– Hyperglycemia

– Hypertension

– Peripheral edema

– Osteoporosis

– Myopathy

– Cataracts

– Glaucoma

– Peptic ulcer disease

– Leukocytosis

– Neutrophilia

– Lymphopenia

– Insomnia

– Irritability

– Mood changes

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/d (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 and 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 and liver and kidney function tests

Cyclophosphamide

1-3 mg/kg/d PO

– GI upset

– Alopecia

– Myelosuppression

– Immunosuppression

– Increased risk of infection

– Infertility in both men (irreversible azoospermia) and women (irreversible amenorrhea)

– Increased risk of malignancies

– Hemorrhagic cystitis

Used only in refractory cases

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 or reduce severity of transfusion reaction

Rituximab

375 mg/m2/wk IV for 4 doses or 1 g followed by another dose of 1 g after 2 weeks

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

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

CBC, complete blood count; GI, gastrointestinal; INN, international nonproprietary name; IVIG, intravenous immunoglobulin; MMP, mycophenolate mofetil; MPA, mycophenolic acid; PO, oral; TPMT, thiopurine methyltransferase.