Introduction
Patients and methods
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 |