Diagnosis and Treatment of Pemphigus

Daisuke Tsuruta; Norito Ishii; Takashi Hashimoto

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

Conclusion

In this article, we reviewed the current state of knowledge concerning the treatment of pemphigus. There is accumulating evidence for the efficacy for various treatment modalities. We began to use newly developed approaches for the treatment of pemphigus. In the coming decade, several new treatment modalities should be available. Treatment algorithms for pemphigus must be continually updated as new data accumulate.

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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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