Anakinra for Inflammatory Pustular Skin Diseases

• INCLUSION CRITERIA

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

1.2 Patients must demonstrate active noninfectious inflammatory pustular skin lesions resembling pustular psoriasis and involving greater than or equal to 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.

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

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

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

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

leukocytes greater than or equal to 3,000/mcL

absolute neutrophil count greater than or equal to1,500/mcL

platelets greater than or equal to 100,000/mcL

creatinine within normal institutional limits OR creatinine clearance greater than or equal to 60 mL/min/1.73 m^2 for patients with creatinine levels above institutional normal.

1.7 Quantiferon tuberculosis (TB) Gold must be performed for screening for mycobacterium tuberculosis infection. However, a tuberculin skin test may be placed if the Quantiferon TB gold test is indeterminate. Patients must have a negative Quantiferon TB Gold (or tuberculin skin test) or evidence of appropriate treatment prior to study entry.

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

EXCLUSION CRITERIA

2.1 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.

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

2.3 History of anakinra use.

2.4 History of phototherapy within 2 weeks prior to study initiation.

2.5 Patients may NOT concurrently be on biologic therapy such as etanercept, adalimumab, alefacept, infliximab, rituximab or rilonacept. 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.

2.6 Subjects who experience a significant flare after discontinuation of a tumor necrosis factor (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.

2.7 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 (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).

2.8 Known diagnosis of Deficiency of the interleukin-1 receptor antagonist (DIRA).

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

2.10 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.

2.11 Patients with active or untreated malignancy-- with the excepti