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Generalized pustular psoriasis in childhood. Report of thirteen cases
1Department of Dermatology, Mayo Clinic, Rochester, MN 55905.
Insights
Generalized pustular psoriasis (GPP) is rare in children, with distinct patterns observed. Topical therapies effectively managed most cases, unlike systemic steroids.
Area of Science:
- Pediatric Dermatology
- Immunodermatology
Background:
- Generalized pustular psoriasis (GPP) is a rare, severe form of psoriasis.
- Pediatric GPP is exceptionally uncommon, with limited case reports.
Observation:
- This study details 13 pediatric cases of GPP.
- Observed patterns included acute Zumbusch (n=7), subacute annular (n=3), and mixed patterns (n=3).
- Zumbusch pattern typically presented in infancy with frequent recurrences.
Findings:
- Preceding eruptions and precipitating factors were common.
- Subacute annular patterns were associated with older children and spontaneous resolution.
- Most pediatric GPP cases showed little chronic morbidity.
Implications:
- Understanding distinct GPP patterns in children aids diagnosis and management.
- Topical therapies appear effective for pediatric GPP.
- Systemic steroids were ineffective, suggesting alternative treatment strategies are needed.
Abstract:
Generalized pustular psoriasis is rare in children. Less than 100 cases have been reported. We describe 13 children with this type of psoriasis. Seven had acute onset of widespread sterile pustules coalescing into lakes of pus with subsequent exfoliation (the Zumbusch pattern). This usually occurred in infancy and was difficult to control; recurrences developed several times per year. Three had the subacute benign annular pattern. They tended to be older and often had resolution within several years. Three had a mixed pattern with Zumbusch flares preceded by an annular or acral pattern. Most patients had an eruption preceding the generalized pustular psoriasis and often had precipitating factors. Generally, generalized pustular psoriasis has little serious chronic morbidity. The condition in most patients was well controlled with topical therapy. Systemic steroids were not helpful.