Diagnosis and Treatment of Pemphigus

Daisuke Tsuruta; Norito Ishii; Takashi Hashimoto

Immunotherapy. 2012;4(7):735-745.

Future Perspective

Anti-TNF-α inhibitors are now being used in the treatment of pemphigus. An anti-TNF-α chimeric monoclonal antibody, infliximab and a fusion protein of the TNF-α receptor and IgG, etanercept, were successfully used to treat pemphigus.^[94,95] Several cholinergic agonists, a Dsg3 peptide and a p38 mitogen-activated protein kinase inhibitor have also been used.^[96]

So far, the etiologic factor for the initial production of autoantibodies in pemphigus remains unclear. After elucidation of these events, targeted therapies can be developed for the treatment of pemphigus.

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Table 1. Classification of pemphigus diseases and their autoantigens.
Disease	Immunoglobulin	Antigen(s)
Pemphigus vulgaris – Mucosal-dominant type – Mucocutaneous type	IgG IgG	Dsg3 Dsg3, Dsg1
Pemphigus vegetans	IgG	Dsg3, Dsg1, Dsc1–3
Pemphigus foliaceus	IgG	Dsg1
Pemphigus erythematosus	IgG	Dsg1
Pemphigus herpetiformis	IgG	Dsg1, (Dsg3), Dsc1–3
Drug-induced pemphigus	IgG	Dsg1
Paraneoplastic pemphigus	IgG	Plakins (plectin, epiplakin, desmoplakins, BP230, envoplakin and periplakin) A2ML1, Dsg3, Dsg1, Dsc
IgA pemphigus – SPD type – IEN type – IgA pemphigus vulgaris – IgA pemphigus foliaceus	IgA IgA IgA IgA	Dsc1 Unknown Dsg3 Dsg1
IgG/IgA pemphigus	IgG and IgA	Dsc1–3
Endemic pemphigus – Brazilian – Tunisian – Colombian	IgG IgG IgG	Dsg1, (Dsg3) Dsg1, Dsg3 Dsg1

Dsc: Desmocollin; Dsg: Desmoglein; IEN: Intra-epidermal neutrophilic IgA dermatosis; SPD: Subcorneal pustular dermatosis.

Box 1. Treatment options for pemphigus vulgaris.
*Mild cases* ▪ Systemic steroid (prednisolone 0.5–0.75 mg/kg/day)
*Moderate–severe cases* ▪ Systemic steroid (prednisolone 0.5–0.75 mg/kg/day) ▪ Change to other type of steroid (e.g., betamethasone, dexamethasone or methylprednisolone, among others) ▪ Add dapsone (50–100 mg/day) ▪ Add mizoribine (150 mg/day once after breakfast)
*Intractable cases* ▪ Systemic steroid (prednisolone 0.75–1.25 mg/kg/day) ▪ Change to other type of steroid (e.g., betamethasone, dexamethasone or methylprednisolone, among others) ▪ Add plasmapheresis (double-filtration plasmapheresis three-times weekly, six- to eight-times) ▪ Add steroid pulse therapy (0.5–1.0 g methylprednisolone for 3 consecutive days) ▪ Add IVIG (400 mg/kg/day for 5 consecutive days) ▪ Add mizoribine (150 mg/day once after breakfast) ▪ Add azathioprine (50–100 mg/day) ▪ Add cyclosporine (3–5 mg/kg/day) ▪ Add mycophenolate mofetil (2 g/day) ▪ Add cyclophosphamide (50 mg/day) ▪ Add rituximab (anti-CD20 antibody; 375 mg/m², once weekly for 4 weeks)

IVIG: Intravenous immunoglobulin.

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Diagnosis and Treatment of Pemphigus

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