Treatment of Autoinflammatory Pustulosis
For autoinflammatory pustulosis in the context of chronic non-bacterial osteitis (CNO), first-line treatment consists of NSAIDs/COX-2 inhibitors, with second-line therapy preferably using intravenous bisphosphonates or TNF-α inhibitors, individualized based on the presence of additional inflammatory features. 1
Understanding the Disease Context
Autoinflammatory pustulosis occurs as part of the sterile bone inflammation spectrum, particularly within CNO (previously termed SAPHO syndrome when accompanied by synovitis, acne, pustulosis, hyperostosis, and osteitis). 1 The condition presents with palmoplantar pustulosis as a key dermatological manifestation alongside bone inflammation, typically affecting the anterior chest wall, vertebrae, mandible, or pelvis in adults. 1
Treatment Algorithm
First-Line Therapy
- Initiate NSAIDs or COX-2 inhibitors as the primary treatment for active CNO with pustulosis. 1
- This addresses both the bone inflammation and associated autoinflammatory features. 1
Second-Line Therapy Selection
When first-line therapy fails, choose between:
- Intravenous bisphosphonates (preferred) for patients with predominantly bone-focused disease. 1
- TNF-α inhibitors for patients with prominent additional inflammatory features (arthritis, sacroiliitis, enthesitis, or severe skin manifestations). 1
Critical caveat: TNF antagonists should be avoided in chronic palmoplantar pustulosis as they may paradoxically exacerbate this specific manifestation. 2 This represents a drug-class effect where anti-TNF therapy can induce paradoxical skin inflammation. 1
Alternative Biologic Approaches
If TNF inhibitors are contraindicated or ineffective:
- IL-17 inhibitors, IL-12/23 inhibitors, or IL-23 inhibitors can be considered for psoriatic skin manifestations in the context of autoinflammatory disease. 2
- Apremilast has demonstrated efficacy in case reports for SAPHO syndrome with pustulosis when other biologics failed or were not tolerated. 3
Systemic Non-Biologic Options
For generalized pustular manifestations:
- Acitretin (0.1-1 mg/kg/day) is particularly effective for pustular disease, with response as early as 3 weeks. 2, 4
- Cyclosporine (initial dose 2.5 mg/kg/day divided twice daily) for severe cases. 2
- Methotrexate for acute generalized pustular presentations. 2
Absolute contraindication: Systemic corticosteroids should be avoided in pustular psoriasis/pustulosis as they risk precipitating erythrodermic psoriasis, generalized pustular psoriasis, or very unstable disease upon withdrawal. 2, 5
Monitoring Requirements
- Regular blood tests, liver function tests, and serum creatinine monitoring are essential depending on the systemic agent used. 2, 5
- Clinical review should assess for disease activity in both bone and skin manifestations. 1, 5
- Monitor for complications including skeletal deformities, compromised joint functionality, and neurovascular entrapment. 1
Special Considerations
Genetic Testing Context
- IL36RN variants are associated with generalized pustular psoriasis and may predict disease severity, though they do not significantly alter treatment response to standard therapies like acitretin. 4
Drug-Induced Pustulosis (AGEP)
If acute generalized exanthematous pustulosis (AGEP) is suspected rather than autoinflammatory pustulosis:
- Immediately discontinue the culprit drug. 6, 7
- Apply topical low-to-moderate potency corticosteroids for mild-moderate cases (10-30% BSA). 6
- Consider systemic corticosteroids (prednisone 0.5-1 mg/kg for 7 days with tapering) only for severe AGEP (>30% BSA). 6
- AGEP is self-limiting and resolves rapidly after drug withdrawal, unlike chronic autoinflammatory pustulosis. 5, 7