摘要
Severe pustular psoriasis von Zumbusch type is a therapeutic challeng e not on ly in adults, but even more in children. We report a 3 1/2- year-old boy who developed a generalized flare of diffusely scattered pustules on erythematous sk in which rapidly progressed to large exuding areas. The clinical presentation an d investigations including histopathological examination of a biopsy and negativ e bacterial cultures were consistent with the diagnosis of pustular psoriasis vo n Zumbusch type. Upon initial treatment withmethylprednisolone, acitretin and an tibiotics the extent of the disease declined. However, several attempts to reduc e the dose of the oral corticosteroid were followed by immediate severe flares. Additional treatmentwith narrowband ultraviolet B (NB-UVB, 311- 313 nm UVB) r esulted in a rapid arrest of disease activity and allowed the corticosteroid to be tapered off. After 10 irradiations the patientwas both off steroid and diseas e free. NB-UVB therapy was subsequently reduced to twice-weekly exposures an d acitretin gradually diminished to a maintenance dose of 0.3 mg kg-1 daily. W e conclude that NB-UVB in conjunction with acitretin is a potent therapeutic r egimen for the treatment of severe pustular psoriasis von Zumbusch type in child hood.
Severe pustular psoriasis von Zumbusch type is a therapeutic challeng e not on ly in adults, but even more in children. We report a 3 1/2- year-old boy who developed a generalized flare of diffusely scattered pustules on erythematous sk in which rapidly progressed to large exuding areas. The clinical presentation an d investigations including histopathological examination of a biopsy and negativ e bacterial cultures were consistent with the diagnosis of pustular psoriasis vo n Zumbusch type. Upon initial treatment withmethylprednisolone, acitretin and an tibiotics the extent of the disease declined. However, several attempts to reduc e the dose of the oral corticosteroid were followed by immediate severe flares. Additional treatmentwith narrowband ultraviolet B (NB-UVB, 311- 313 nm UVB) r esulted in a rapid arrest of disease activity and allowed the corticosteroid to be tapered off. After 10 irradiations the patientwas both off steroid and diseas e free. NB-UVB therapy was subsequently reduced to twice-weekly exposures an d acitretin gradually diminished to a maintenance dose of 0.3 mg kg-1 daily. W e conclude that NB-UVB in conjunction with acitretin is a potent therapeutic r egimen for the treatment of severe pustular psoriasis von Zumbusch type in child hood.
