Inclusion Criteria

- INCLUSION CRITERIA

Females and males, aged greater than or equal to 18.

Patients must demonstrate active noninfectious inflammatory pustular skin lesions
resembling pustular psoriasis and involving >= 5% total body surface area, or palmoplantar
involvement. Conditions may include, but are not be limited to, pustular psoriasis,
Sneddon-Wilkinson disease, subcorneal pustular dermatosis, reactive arthritis,
palmoplantar pustulosis, acrodermatitis continua of Hallopeau and palmoplantar pustular
psoriasis.

Patients must have histopathologic confirmation of epidermal neutrophilic pustular skin
disease.

If taking immunosuppressants, retinoids or anti-neutrophil therapy, participants must
maintain stable doses of these medications during the 2 weeks prior to study initiation.

Patients must have stable topical medication regimen for 2 weeks prior to study initiation

Patients must have normal organ and marrow function as defined below:

leukocytes > 3,000/mcL

absolute neutrophil count > 1,500/mcL

platelets > 100,000/mcL

creatinine within normal institutional limits OR creatinine clearance > 60 mL/min/1.73 m2
for patients with creatinine levels above institutional normal.

Patients must have either (1) a negative PPD test per CDC guidelines and no evidence of
active TB on chest radiograph at the time of enrollment, or (2) a positive PPD with no
evidence of active TB by history or on chest radiograph at the time of enrollment AND
either past or present treatment with adequate therapy for at least one month prior to
first dose of study medication.

Patients must be able to understand and sign a written informed consent document and
complete study-related procedures and questionnaires.

EXCLUSION CRITERIA

Enrollment in any other investigational treatment study or use of an investigational
agent, or has not yet completed at least 3 half-lives since ending another investigational
device or drug trial.

History of treatment with canakinumab within the 12 months prior to study initiation.

History of anakinra use.

History of phototherapy within 2 weeks prior to study initiation.

Patients may NOT concurrently be on biologic therapy such as etanercept, adalimumab,
alefacept, infliximab, rituximab or rilonacept (Appendix A). If there is a history of use
of biologic agents, there must be a washout period of at least 3 half-lives prior to study
initiation.

Subjects who experience a significant flare after discontinuation of a TNF inhibitor as
part of this study that requires urgent medical management or hospitalization, or in the
estimation of the principal investigator poses excessive risk to the patient to enter the
study.

Other defined dermatologic conditions which may include pustules as part of the clinical
presentation, but which clinically and/or histologically do not resemble pustular
psoriasis. Examples include, but are not limited to acute generalized exanthematous
pustulosis (AGEP, a drug-induced pustular dermatosis typically caused by beta-lactam
antibiotics, tetracyclines, oral antifungals and other drugs), bacterial or fungal
folliculitis, cutaneous candidiasis, tinea pedis, tinea corporis, neutrophilic eccrine
hidradenitis or eosinophilic pustular folliculitis (Ofuji syndrome).

Known diagnosis of DIRA.

History of allergic reactions attributed to compounds of similar chemical or biologic
composition to anakinra or other agents used in study. Known hypersensitivity to CHO-cell
derived biologics or any components of anakinra.

Treatment with a live virus vaccine during the 3 months prior to baseline visit. No live
vaccines will be allowed throughout the course of this study.

Pregnant or lactating females.

Patients with active or untreated malignancy-- with the exception of cutaneous basal or
squamous cell carcinomas, or in situ cervical carcinoma-- are ineligible because of the
immunomodulating effects of anakinra. The risk of recurrent malignancy secondary to this
drug is unknown.

Presence of active infection. History of exposure to TB (positive PPD) who have not been
treated with a TB prophylaxis regimen for at least one month.

Chest x-ray demonstrating pleural scarring and/or calcified granuloma consistent with
prior or current untreated TB.

History of chronic or recurrent infection including but not limited to HIV, hepatitis B or
hepatitis C.

Individuals with severe or uncontrolled recurrent cutaneous infections who are considered
at elevated risk for serious infection on anakinra therapy will be excluded per physician
discretion.

Presence of other known significant autoimmune or inflammatory disease. Examples include
major chronic infectious/inflammatory/immunologic diseases such as systemic lupus
erythematosus, rheumatoid arthritis, Sjogren?s syndrome and periodic fever syndromes.

Other immunoregulatory or immunodeficiency diseases, such as multiple sclerosis.

Individuals with life-threatening or disabling inflammation of the eyes, gut or joints
requiring urgent or immediate medical attention, or at the physician?s discretion.

Subjects for whom there is concern about compliance with the protocol procedures.

Uncontrolled intercurrent illness including, but not limited to, ongoing or active
infection, uncontrolled or unmonitored psychiatric illness/social situations, or history
of congestive heart failure, unstable angina pectoris or medically significant cardiac
arrhythmia that would limit compliance with study requirements.

Presence of other severe acute or chronic medical or psychiatric condition, or significant
laboratory abnormality requiring further investigation that may cause undue risk for the
subject?s safety, inhibit protocol participation, or interfere with interpretation of
study results, and in the judgment of the investigator would make the subject enrollment
inappropriate.

The effects of anakinra on the developing human fetus are unknown. Women of childbearing
potential and men must agree to use adequate contraception (hormonal or barrier method of
birth control or abstinence) prior to study entry and for the duration of study
participation. Should a woman become pregnant or suspect she is pregnant while she or her
partner is participating in this study, she should inform her treating physician
immediately. Females of childbearing potential must have a negative urine pregnancy test
at screening. Females must also have a negative serum pregnancy test at baseline and prior
to performance of any radiologic procedure or administration of study medication and
during each NIH visit. Lactating mothers will discontinue breastfeeding prior to study
enrollment.