Ichthyosiform erythroderma associated with generalized pustulosis
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Published on: October 12, 2012
J A Langtry1, M M Carr, F A Ive
1Department of Dermatology, Sunderland Royal Hospital, London, U.K.
The British Journal of Dermatology
|May 15, 1998
Insights
Two boys born as collodion babies developed ichthyosis and pustulosis. Oral retinoids effectively treated their skin conditions, similar to generalized pustular psoriasis.
Area of Science:
- Dermatology
- Genetics
- Pediatrics
Background:
- Collodion babies represent a rare presentation of severe congenital ichthyosis.
- Ichthyosis and pustulosis are distinct but can co-occur in certain genetic disorders.
- Generalized pustular psoriasis is a severe inflammatory skin condition characterized by sterile pustules.
Observation:
- Two unrelated male infants presented as collodion babies at birth.
- Both patients subsequently developed non-bullous ichthyosiform erythroderma.
- Recurrent flares of generalized sterile pustulosis, resembling generalized pustular psoriasis, were observed.
Findings:
- The clinical presentation mimicked aspects of generalized pustular psoriasis in both cases.
- Histopathological examination confirmed non-bullous ichthyosiform erythroderma and sterile pustules.
- Both patients demonstrated a significant clinical improvement with oral retinoid therapy.
Implications:
- This case series highlights a potential overlap between severe ichthyosis and pustular conditions.
- Oral retinoids may be a viable therapeutic option for this specific phenotype.
- Further research into the genetic basis and pathogenesis of this condition is warranted.
Abstract:
Two unrelated boys are described who were born as collodion babies and subsequently developed non-bullous ichthyosiform erythroderma and flares of generalized sterile pustulosis, similar to generalized pustular psoriasis. Both patients had a good response to treatment with oral retinoids.
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