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Chapter 43: Intercellular IgA Dermatosis (IAD)/IgA Pemphigus

Takashi Hashimoto

INTERCELLULAR IgA DERMATOSIS (IAD)/IgA PEMPHIGUS

Therapeutic Approach

Intercellular IgA dermatosis (IAD), which is also called as IgA pemphigus, is a rare autoimmune bullous disease, characterized by tissue-bound and circulating IgA autoantibodies against epidermal keratinocyte cell surfaces. IAD is divided into two major subtypes, subcorneal pustular dermatosis (SPD) type and intraepidermal neutrophilic IgA dermatosis (IEN) type, with minor subtypes of IgA pemphigus vegetans, IgA pemphigus foliaceus, IgA pemphigus vulgaris, and undetermined type. Therapies consist of both anti-inflammatory and immunosuppressive medications. However, consensus for the treatments for IAD has not been obtained. See Table 43-1.

Table 43-1Intercellular IgA Dermatosis/IgA Pemphigus Treatment Table
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Table 43-1 Intercellular IgA Dermatosis/IgA Pemphigus Treatment Table
MEDICATION NAME INDICATION MECHANISM OF ACTION DOSING ADVERSE EFFECTS SUGGESTED MONITORING LEVEL OF EVIDENCE (REFERENCE)
Topical Therapy
Corticosteroids All subtypes Immunosuppressive, anti-inflammatory 0.12% Betamethasone valerate ointment, 0.05% difluprednate ointment; apply BID Skin fragility, telangiectasia None III1,2
Systemic Therapy
Corticosteroids All subtypes Immunosuppressive, anti-inflammatory 10-30 mg as prednisolone QD-BID GI upset, diabetes mellitus, hypertension, osteoporosis CBC, CMP, lipid panel (every 3 mo) III1-4
Dapsone/DDS (diaminodiphenylsulfone) All subtypes Anti-inflammatory 25-50 mg BID GI upset, anemia, drug eruption CBC, CMP (every 3 mo) III1,2,5-9
Doxycycline All subtypes Anti-inflammatory 100 mg BID GI upset, increased liver enzymes CBC, CMP (every 3 mo) IB9
Minocycline All subtypes Anti-inflammatory 50-100 mg BID GI upset, increased liver enzymes, skin pigmentation CBC, CMP (every 3 mo) IB9
Azithromycin All subtypes Anti-inflammatory 500 mg BID-TID GI upset, increased liver enzymes CBC, CMP (every 3 mo) IV10
Colchicine All subtypes Anti-inflammatory 1 mg TID GI upset, anemia, increased liver enzymes, kidney damage CBC, CMP (every 3 mo) IV11,12
Azathioprine All subtypes Immunosuppressive 50-100 mg BID Infections, increased liver enzymes CBC, CMP (every 2 mo) IV13
Mycophenolate mofetil All subtypes Immunosuppressive 250-500 mg BID Infections, increased liver enzymes CBC, CMP (every 2 mo) IV14,15
Methotrexate All subtypes Immunosuppressive 6 mg once weekly Infections, increased liver enzymes CBC, CMP (every 2 mo) IV4
Retinoids All subtypes Anti-inflammatory Etretinate (10-30 mg), Isotretinoin (10-20 mg), Acitretin (10-20 mg), Alitretinoin (5-10 mg) BID Teratogenic, skin dryness, nausea, increased lipid and liver enzymes CBC, CMP, lipid panel (every 3 mo), pregnancy test (at starting time) IV9,16-20
Adalimumab All subtypes Anti-inflammatory 80 mg sc every 2 wk Various infections, others CBC, CMP (every 3 mo) IV15
Procedural Therapy
Intravenous immunoglobulin All subtypes Various effects 0.4 g/kg DIV for 5 d Headache, fever, thrombocytopenia CBC, CMP (every 2 wk) IV21
Plasma exchange/Plasmapheresis All subtypes Removal of autoantibodies Twice weekly Thrombocytopenia CBC, CMP (every 2 wk) IV9

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 ...

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