Generalized Pustular Psoriasis in Childhood

• Published in: Pediatric dermatology 2010-07, Vol.27 (4), p.349-354
• Main Authors: De Oliveira, Sarah Toyomi, Maragno, Luciana, Arnone, Marcelo, Fonseca Takahashi, Maria Denise, Romiti, Ricardo
• Format: Article
• Language: English
• Subjects: Anti-Bacterial Agents - therapeutic use; Bacterial Infections - diagnosis; Bacterial Infections - drug therapy; Biological and medical sciences; Biological Products - therapeutic use; Bullous diseases of the skin; Child; Child, Preschool; Dermatology; Fatal Outcome; Follow-Up Studies; General aspects; Humans; Immunosuppressive Agents - therapeutic use; Infant, Newborn; Male; Medical sciences; Nail Diseases - drug therapy; Nail Diseases - pathology; Phototherapy; Psoriasis - drug therapy; Psoriasis - pathology; Recurrence; Retinoids - therapeutic use; Retrospective Studies; Scalp Dermatoses - drug therapy; Scalp Dermatoses - pathology; Shock, Septic - diagnosis; Shock, Septic - drug therapy; Sulfasalazine - therapeutic use; Treatment Outcome
• ISSN: 0736-8046; 1525-1470
• DOI: 10.1111/j.1525-1470.2010.01084.x

:  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.