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Chronic Cutaneous Pustulosis Due to a 175-kb Deletion on Chromosome 2q13:  Excellent Response to Anakinra

Cristina N. Brau-Javier, MD; Jose Gonzales-Chavez, MD; Jorge R. Toro, MD
Arch Dermatol. 2012;148(3):301-304. doi:10.1001/archdermatol.2011.2857.
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Deficiency of interleukin 1 receptor antagonist (DIRA) is a newly described autosomal recessive autoinflammatory disease in which absence of interleukin 1 (IL-1) receptor antagonist allows unopposed IL-1 activation, resulting in life-threatening systemic inflammation with prominent skin and bone involvement. We present a case of chronic cutaneous pustulosis treated with anakinra, a recombinant IL-1 receptor antagonist, resulting in rapid and significant clinical improvement.

Department of Dermatology, University of Puerto Rico, Puerto Rico (Drs Brau-Javier and Gonzales-Chavez); and Division of Cancer Epidemiology and Genetics, Genetic Epidemiology Branch, National Cancer Institute, Bethesda, Maryland (Dr Toro)Correspondence: Jorge R. Toro, MD, Division of Cancer Epidemiology and Genetics, National Cancer Institute, 6120 Executive Blvd, Executive Plaza S, Room 7012, Rockville, MD 20892 (toroj@mail.nih.gov).

Accepted for Publication: November 24, 2011.

Author Contributions: Dr Toro had full access to all the data in the study and all authors take responsibility for the integrity of the data. Study concept and design: Brau-Javier, Gonzales-Chavez, and Toro. Acquisition of data: Brau-Javier, Gonzales-Chavez, and Toro. Analysis and interpretation of data: Brau-Javier, Gonzales-Chavez, and Toro. Drafting of the manuscript: Brau-Javier, Gonzales-Chavez, and Toro. Critical revision of the manuscript for important intellectual content: Brau-Javier, Gonzales-Chavez, and Toro. Administrative, technical, and material support: Brau-Javier, Gonzales-Chavez, and Toro. Study supervision: Brau-Javier, Gonzales-Chavez, and Toro.

Financial Disclosure: None reported.

Funding/Support: This study was supported in part by federal funds from the Intramural programs (Division of Cancer Epidemiology and Genetics) of the National Cancer Institute.

Disclaimer: The content of this publication does not necessarily reflect the views or policies of the Department of Health and Human Services, nor does mention of trade names, commercial products, or organizations imply endorsement by the US government.

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Figure 1. A, Abdominal distention and erythematous plaques with overlying pustules; B, close-up of abdominal lesions revealing pustules.

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Figure 2. Psoriasiform dermatitis with a subcorneal pustule (hematoxylin-eosin, original magnification ×4).

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Figure 3. Significant improvement in abdominal lesions at 10 days of follow-up after anakinra treatment.




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