title: Successful use of acitretin in conjunction with narrowband ultraviolet B phototherapy in a child with severe pustular psoriasis, von Zumbusch type.
abstract: Severe pustular psoriasis von Zumbusch type is a therapeutic challenge not only 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 skin which rapidly progressed to large exuding areas. The clinical presentation and investigations including histopathological examination of a biopsy and negative bacterial cultures were consistent with the diagnosis of pustular psoriasis von Zumbusch type. Upon initial treatment with methylprednisolone, acitretin and antibiotics the extent of the disease declined. However, several attempts to reduce the dose of the oral corticosteroid were followed by immediate severe flares. Additional treatment with narrowband ultraviolet B (NB-UVB, 311-313 nm UVB) resulted in a rapid arrest of disease activity and allowed the corticosteroid to be tapered off. After 10 irradiations the patient was both off steroid and disease free. NB-UVB therapy was subsequently reduced to twice-weekly exposures and acitretin gradually diminished to a maintenance dose of 0.3 mg kg(-1) daily. We conclude that NB-UVB in conjunction with acitretin is a potent therapeutic regimen for the treatment of severe pustular psoriasis von Zumbusch type in childhood.
authors.affiliation: Department of Paediatrics, Division of General Paediatrics, Vienna Medical University, Währinger Gürtel 18-20, A-1090 Vienna, Austria. tamara.kopp@akh-wien.ac.at
authors.forename: T
authors.initials: T
authors.lastname: Kopp
authors.forename: F
authors.initials: F
authors.lastname: Karlhofer
authors.forename: Z
authors.initials: Z
authors.lastname: Szépfalusi
authors.forename: A
authors.initials: A
authors.lastname: Schneeberger
authors.forename: G
authors.initials: G
authors.lastname: Stingl
authors.forename: A
authors.initials: A
authors.lastname: Tanew
mesh_terms: D017255:Acitretin / Q000627:therapeutic use*; D002675:Child, Preschool; D003131:Combined Modality Therapy; D006801:Humans; D007641:Keratolytic Agents / Q000627:therapeutic use*; D008297:Male; D011565:Psoriasis / Q000188:drug therapy* / Q000473:pathology / Q000532:radiotherapy*; D014467:Ultraviolet Therapy
chemical_list: D007641:Keratolytic Agents; D017255:Acitretin