title: Generalized pustular psoriasis in childhood.
abstract: Generalized pustular psoriasis is a rare form of psoriasis consisting of a generalized eruption of sudden onset with erythema and sterile pustules. In children, generalized pustular psoriasis is even more uncommon and may present as a severe and potentially life-threatening disorder. In this study, we present demographics, clinical aspects, treatment response, and follow-up of seven children with generalized pustular psoriasis. Retrospective study reviewing the records of seven children with generalized pustular psoriasis including age, gender, age of onset, presence of scalp and nail involvement, family history, concomitant diseases, precipitating factors, treatment modalities, and outcome. Age of first symptoms ranged from 1 month to 11 years. All patients received systemic retinoids at one time of the follow-up period. Other treatment modalities included immunosuppressive drugs, biologics, phototherapy, and sulfasalazine. Two patients presented with severe constitutional illness, secondary infection and septic shock, including one fatal outcome. All further cases have remained free of recurrences for a mean period of up to 3 years. In our study, generalized pustular psoriasis presented a wide clinical spectrum in children ranging from mild, asymptomatic outbreaks to more severe, life-threatening episodes. One fatality was observed. Children generally responded well to systemic retinoids. Further studies and long-term follow-up periods are needed to define potential trigger factors, efficacy and safety of different treatment modalities in children with generalized pustular psoriasis.
authors.affiliation: Department of Dermatology, University of São Paulo, São Paulo, Brazil. emaildasarah@yahoo.com
authors.forename: Sarah Toyomi
authors.initials: ST
authors.lastname: de Oliveira
authors.forename: Luciana
authors.initials: L
authors.lastname: Maragno
authors.forename: Marcelo
authors.initials: M
authors.lastname: Arnone
authors.forename: Maria Denise
authors.initials: MD
authors.lastname: Fonseca Takahashi
authors.forename: Ricardo
authors.initials: R
authors.lastname: Romiti
mesh_terms: D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D001424:Bacterial Infections / Q000175:diagnosis / Q000188:drug therapy; D001688:Biological Products / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D017809:Fatal Outcome; D005500:Follow-Up Studies; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007231:Infant, Newborn; D008297:Male; D009260:Nail Diseases / Q000188:drug therapy / Q000473:pathology; D010789:Phototherapy; D011565:Psoriasis / Q000188:drug therapy* / Q000473:pathology*; D012008:Recurrence; D012176:Retinoids / Q000627:therapeutic use; D012189:Retrospective Studies; D012536:Scalp Dermatoses / Q000188:drug therapy / Q000473:pathology; D012772:Shock, Septic / Q000175:diagnosis / Q000188:drug therapy; D012460:Sulfasalazine / Q000627:therapeutic use; D016896:Treatment Outcome
chemical_list: D000900:Anti-Bacterial Agents; D001688:Biological Products; D007166:Immunosuppressive Agents; D012176:Retinoids; D012460:Sulfasalazine