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Chapter 38: Pemphigus

Christoph T. Ellebrecht; Aimee S. Payne

PEMPHIGUS

Therapeutic Approach

Pemphigus is a group of intraepidermal blistering diseases characterized by autoantibodies to the keratinocyte adhesion proteins desmoglein 3 (DSG3) and desmoglein 1 (DSG1), which cause flaccid blisters and erosions of the skin and/or mucous membranes. Major subtypes are pemphigus vulgaris (PV), which includes pemphigus vegetans and pemphigus foliaceus (PF), which includes pemphigus erythematosus. Therapy is based on the severity of disease and patient comorbidities. In the authors’ experience, topical therapy and/or doxycycline can be initiated for mild activity at disease onset, and systemic therapy with corticosteroids and rituximab are first-line therapy for moderate to severe disease, as well as any extent of disease that is relapsed or refractory to initial treatment. All theraipes for pemphigus in the table below are used off-label, with the exception of corticosteroids and rituximab.

Levels of evidence are based on the Journal of the American Academy of Dermatology guidelines: level IA evidence includes evidence from meta-analysis of randomized controlled trials; level IB evidence includes evidence from ≥1 randomized controlled trial; level IIA evidence includes evidence from ≥1 controlled study without randomization; level IIB evidence includes evidence from ≥1 other type of experimental study; level III evidence includes evidence from nonexperimental descriptive studies, such as comparative studies, correlation studies, and case control studies; and level IV evidence includes evidence from expert committee reports or opinions or clinical experience of respected authorities, or both. See Table 38-1.

Table 38-1Pemphigus Treatment Table
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Table 38-1 Pemphigus Treatment Table
MEDICATION NAME INDICATION MECHANISM OF ACTION DOSING ADVERSE EFFECTS SUGGESTED MONITORING LEVEL OF EVIDENCE (REFERENCE)
Topical Therapy
Corticosteroids PV/PF Anti-inflammatory via inhibition of NF Kappa B, increases transcription of DSG3 and other adhesion molecules Apply QD-BID Skin atrophy, striae, pigmentary changes, telangiectasia, purpura, acne N/A IV1
Tacrolimus, pimecrolimus PV/PF Calcineurin inhibitor, decreases T cell signaling and IL-2 transcription Apply QD-BID N/A (topical) systemic therapy carries black box warning for lymphoma N/A N/A2
Systemic Therapy
Corticosteroids PVa/PFa Global immunosuppressant, anti-inflammatory via inhibition of NF Kappa B, increases transcription of DSG3 and other adhesion molecules

Oral: 0.5-1.5 mg/kg/d prednisone equivalent until disease control is achieved, consider BID dosing, taper duration based on response.

Refractory cases: IV pulse therapy (eg, 1 g/d methylprednisolone IV for 3-5 d) until disease control is achieved, taper duration based on response

 Hyperglycemia, hypertension, adrenal insufficiency, osteoporosis, osteonecrosis, peptic ulcer, cataract, glaucoma, psychiatric abnormalities, infections, myopathy, acne, delayed wound healing

Osteoporosis: Counseling and baseline T score if anticipated duration >3 mo.

Tuberculosis screening, Pneumocystis prophylaxis if anticipated duration >1 month and dose >30 mg/d prednisone equivalent.

Rule out adrenal insufficiency prior to discontinuation.

For IV pulse therapy protocols: cardiac, electrolyte, blood glucose monitoring on days of infusion

Oral: IB3, 4

IV pulse therapy: IV1,5,6
Rituximab PVa/PF CD20+ B cell depletion (spares ...

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