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Background: The immune abnormalities underlying the ichthyoses are poorly understood., Objective: To determine the immunophenotype of an ichthyosis resulting from mutations in the spectrin repeat 6 (SR6) domain of desmoplakin gene (DSP) and target therapy on the basis of molecular pathogenesis., Methods: Immunophenotyping was performed by using the blood and skin of a girl with SR6 region DSP mutations causing erythroderma/ichthyosis and cardiomyopathy., Results: On the basis of the discovery of T helper 1 and T helper 17/interleukin 23 skewing in the skin and T helper 17/interleukin 22 skewing in blood, ustekinumab therapy was initiated. Ustekinumab was also administered to a boy with an SR6 region DSP mutation and ichthyosis without cardiomyopathy. Both children responded despite previous poor responses to immunosuppressants and retinoids., Limitations: Small number of patients and immunophenotyping in only 1 patient., Conclusion: An understanding of the molecular basis of inflammation in rare cutaneous disorders can lead to targeted therapy, which promises to be more beneficial than broad immunosuppressants., (Copyright © 2017 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)

Background: The prevalence and pathogenesis of rosacea is uncertain. Previously, studies used varying definitions of disease and have not explored the relationship of its prevalence to ultraviolet (UV) exposure or photodamage., Objectives: We investigated the prevalence of papulopustular rosacea (PPR) and its relationship to UV radiation exposure in 1000 randomly selected Irish individuals., Methods: A total of 1000 individuals (500 with low UV exposure and 500 with high UV exposure) were examined. PPR was diagnosed using a standardized definition and photodamage was assessed using a photodamage scale., Results: The prevalence of PPR was 2.7%. PPR prevalence was not significantly related to photodamage or UV exposure., Limitations: The power to compare UV exposure among those with and without PPR was limited., Conclusions: PPR prevalence in Ireland was 2.7%. UV radiation exposure does not appear to affect the prevalence of PPR., (Copyright (c) 2009 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.)

Pyoderma gangrenosum (PG) is rare in infants. There have been 12 cases of PG in infants (<12 months old) reported in the past 25 years, to our knowledge. Six of these cases have been successfully controlled with systemic steroids, and one case with topical steroids alone. We report a case of an 8-month-old infant whose PG was aggressive and unresponsive to systemic steroids. Adjuvant treatment with cyclosporine was required to achieve healing. We review the previous cases of infantile PG and the therapeutic options in this age group.