Table 1 Clinical and laboratory findings of the patient cohort
From: Bullous pemphigoid in infants: characteristics, diagnosis and treatment
Age (months)/Gender | Extent of Disease/Hands/Feet (HF) | OM | DIF/IIF/Immunoblot | ELISA | WBC (Eos in%) Thrombocytes | Treatment | Time Until Remission/Relapse/Duration of Treatment | Special aspects | |
|---|---|---|---|---|---|---|---|---|---|
1) | 3/M | Generalized | + | DIF: IgG, C3 (BM) | Anti BP180 | 10.4 × 109/l (10) | a) Prednisolone | Initially rapid response with disease control | Family history of atopy |
HF+ | IIF: IgG (BR) | 136 U/ml | At relapse: | 2 mg/kg/d → 1 mg/kg/d | Relapse within 2 weeks after diagnosis on systemic prednisolone (2 mg/kg) and during respiratory tract infection | Rotavirus vaccine | |||
IB: 180kD pos. | (norm < 9) | 54 × 109/l (52) | b) Dapsone 2 mg/kg/d | Slow response after relapse, need for multiple medications | 4 weeks prior | ||||
At relapse: | Tc >1000 × 109/l | c) IVIG 1 g/kg × 3 | Response to dapsone after 2.5 weeks | ||||||
Anti BP180 | d) MMF (2× 600 mg/m2/ d) | Duration of treatment: 8 months | |||||||
189 U/ml | |||||||||
Anti BP230 neg. | |||||||||
2) | 3/M | Localized with few disseminated lesions HF+ | - | DIF: IgG, C3 (BM) | Anti BP180 | 16.1 × 109/l (23) | a) Topical Prednicarbate | Good response to topical treatment within days | |
IIF: IgG (BR) | 90 U/ml | (mid-potency corticosteroid) | No relapse | ||||||
IB: 180kD pos. | (norm < 9) | Duration of treatment: 4 weeks | |||||||
Anti BP230 neg. | |||||||||
3) | 4/M | Generalized | - | DIF: IgG, C3 (BM) | Anti BP180 | 23.4 × 109/l (20) | a) Prednisolone | Complete remission within 1 week | Vaccination 4 weeks prior |
HF+ | IIF: IgG (BR) | 156 U/ml | 2 mg/kg/d → 1 mg/kg/d | Weaning of steroids within 3 months | (DPTP, HiB, HepB, Rotavirus) | ||||
IB: 180kD pos. | (norm < 9) | b) Dapsone 1.5 mg/kg/d | No relapse | ||||||
Anti BP230 neg. | Duration of treatment: 6 months | ||||||||
4) | 3/F | Generalized | - | DIF: IgG, C3 (BM) | Anti BP180 | 25.1 × 109/l (13) | a) Prednisolone | Slow response to prednisolone 1 mg/kg | Rotavirus vaccine |
HF+ | IIF: IgG (BR) | 125U/ml | Tc 860 × 109/l | 2 mg/kg/d → 1 mg/kg/d | Rapid response to oral betamethasone 0.3 mg/kg/d | 4 weeks prior | |||
IB: 180kD pos. | (norm < 9) | b) Systemic betamethasone | No relapse upon glucocorticoid tapering | Arterial hypertension | |||||
Anti BP230 neg. | 0.3 mg/kg/d | Complete remission under dapsone 0.5 mg/kg/d | Myocardial hypertrophy | ||||||
c) Dapsone | Treatment ongoing | → Propranolol | |||||||
1 mg/kg/d → 0.5 mg/kg/d | |||||||||
5) | 7/M | Generalized | - | DIF: IgG, C3 (BM) | Anti BP180 | 27.3 × 109/l (9) | a) Prednisolone 1 → 0.5 mg/kg/d | Rapid response to oral betamethasone | |
HF+ | IIF: IgG (BR) | 154 U/ml | Tc 599 × 109/l | b) Systemic betamethasone | Full remission after 2 months | ||||
IB: 180kD pos. | (norm < 9) | 0.4 mg/kg/d → 0.2 mg/kg/d | No relapse | ||||||
Anti BP230 neg. | c) Dapsone 0.5 mg/kg/d | Treatment ongoing |