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Table 1 Summary of the patient history with clinical presentation and drug history, as well as the diagnostic analyses performed in all 3 patients reported here

From: Systemic retinoids for treatment of recalcitrant IgA pemphigus

No.

Sex

Agea

Clinical presentation

DIF

IIF ME

IB

Gammopathy

Previous treatment, dosage and durationb

Systemic prednisolone dosage

Retinoid dosage

Side effects

1

F

63

Solid flaccid blisters primarily in intertriginous areas (subcorneal pustular dermatosis type)

Subcorneal IgA IC

IC IgA

DSC3

Monoclonal IgGκ, BJ proteins negative

Azathioprine (2.5 mg/kg) (15 mo) colchicine (1.5 mg/d) (4 mo) acitretin (0.3 mg/kg) (3 mo) anakinra (1 g) (once) methotrexate (15 mg/we) (3 mo) c

5 mg

Alitretinoin 30 mg/d (since 3 yrs.)

Acitretin: hair loss, extreme dryness of skin and mucosa, hyperlipoproteinaemia, reduction of visual acuity Alitretinoin: hyperlipoproteinaemia

2

F

57

Multiple, annular sterile pustules on erythematous plaques on the trunk (subcorneal pustular dermatosis type)

Subcorneal IgA IC

IC IgA

DSC3

Not found

Azathioprine (1.5 mg/kg) (3 mo, hepatopathy MMF 2 g (6 mo) MMF 2 g + dapsone (1 mg/kg) (6 mo) colchicine (3 mg/d) (2 yrs.) acitretin (0.5 mg/kg) (4 mo)

7.5/ 10 mg

Alitretinoin 30 mg/d (since 2 yrs.)

Acitretin: hair loss, dizziness, dryness of mucosa

None under alitretinoin

3

F

57

Disseminated single and confluent sterile pustules on erythematous plaques on the trunk and les on the extremities (subcorneal pustular dermatosis type)

Subcorneal IgA IC

IC IgA

n.a.

Monoclonal IgAλ, BJ proteins negative

Cyclophosphamide + fortecortin (cum. 11 mg) (11 months) azathioprine (2 mg/kg) (3 mo) thalidomide (100 mg/d) (3 mo) ciclosporine (̴ 2 mg/kg) (3.5 yrs.) ciclosporine (̴ 2 mg/kg) + acitretin (0.5 mg/d) (3 mo) acitretin (0.5 mg/d) + dapsone (1.5 mg/kg) (6.5 yrs)

0 mg

Acitretin 10 mg/d (since 6 yrs.)

Acitretin: discrete hyperlipoproteinaemia

  1. aAge at time of diagnosis; BJ, Bence Jones light chains, cum, cumulative dosage, DSC desmocollin, IC intercellular, ME monkey esophagus, MMF mycophenolate mofetil, mo months, κ kappa, λ lambda; n.a. not available, yrs. years
  2. bThe previous treatments are presented in chronological order
  3. cPatient 1 has glucose-6-phosphate-dehydrogenase deficiency, thus dapsone was not a treatment option