Treatment of Panniculitis
Treatment of panniculitis must be guided by the underlying etiology, with alpha-1 antitrypsin (AAT) deficiency-associated panniculitis requiring augmentation therapy, connective tissue disease-associated panniculitis requiring immunosuppression, and vasculitis-associated panniculitis requiring cyclophosphamide plus high-dose glucocorticoids. 1
Diagnostic Workup Before Treatment
Before initiating therapy, obtain a deep excisional biopsy with adequate subcutaneous tissue—superficial punch biopsies will miss the pathology and lead to misdiagnosis 1, 2. The biopsy must include sufficient fat to distinguish septal from lobular patterns and identify vasculitis 3.
Test all patients with severe or necrotizing panniculitis for alpha-1 antitrypsin deficiency, as this specific etiology requires targeted augmentation therapy rather than empiric immunosuppression 1, 2. Additionally, rule out systemic vasculitis, malignancy, inflammatory bowel disease, and connective tissue diseases through appropriate laboratory studies and imaging 2.
Treatment by Specific Etiology
Alpha-1 Antitrypsin Deficiency-Associated Panniculitis
Augmentation therapy with purified human AAT or fresh frozen plasma is the most effective treatment, as it restores both plasma and local tissue AAT levels 1. This represents the definitive therapy for AAT deficiency-associated panniculitis, which typically presents as painful nodules on thighs/buttocks that ulcerate and drain clear, oily fluid 4.
- Dapsone can be used alone in less severe cases or combined with augmentation therapy for enhanced benefit 1
- Provide antismoking counseling and screen family members, as smoking further reduces functional AAT capacity and increases risk 1
- Liver transplantation has achieved permanent cure in severe cases by restoring normal plasma AAT production 1
Connective Tissue Disease-Associated Panniculitis
For lupus panniculitis, antimalarials are the initial treatment of choice and should be started during the active inflammatory phase before scarring and atrophy develop 5. Treatment is most effective when initiated early, as the disease can progress to permanent tissue damage 5.
For dermatomyositis-associated panniculitis, use corticosteroids combined with steroid-sparing immunosuppressive agents as first-line therapy 5. Systemic therapy is typically required due to the deep location of inflammation 5.
Vasculitis-Associated Panniculitis
For polyarteritis nodosa with cutaneous panniculitis, treat with cyclophosphamide plus high-dose glucocorticoids 1. When disease is severe, initiate with intravenous pulse glucocorticoids rather than high-dose oral steroids 1.
- In patients unable to tolerate cyclophosphamide, substitute alternative non-glucocorticoid immunosuppressive agents 1
- Nodular thrombophlebitis requires evaluation for coagulopathy, Behçet's disease, or Buerger's disease 3
Malignancy-Associated Panniculitis
Address both the hemophagocytic lymphohistiocytosis and the underlying neoplasm simultaneously 1. Cyclosporin A has demonstrated efficacy in clonal cytophagic histiocytic panniculitis and subcutaneous panniculitis-like T-cell lymphoma with hemophagocytic features 1.
For panniculitis-associated hemophagocytic syndrome refractory to standard therapy, ruxolitinib combined with dexamethasone has shown effectiveness by targeting the JAK-STAT pathway and reducing inflammatory cytokine release 6.
Idiopathic Panniculitis
After excluding all specific etiologies through comprehensive workup, treatment typically involves anti-inflammatory and immunosuppressive agents, with systemic corticosteroids combined with methotrexate or thalidomide showing benefit in case reports 7, 8.
Monitoring and Follow-Up
Perform follow-up abdominal vascular imaging in patients with severe panniculitis and prior abdominal involvement who become clinically asymptomatic 1. For patients with peripheral motor neuropathy secondary to panniculitis, use serial neurologic examinations rather than repeated electromyography/nerve conduction studies 1.
Critical Pitfalls to Avoid
- Never rely on superficial biopsies—inadequate depth is the most common diagnostic error leading to missed diagnoses 1, 2
- Do not assume idiopathic disease without testing for AAT deficiency, as this treatable cause requires specific augmentation therapy rather than empiric immunosuppression 1, 2
- Panniculitis can be lethal, particularly when associated with cirrhosis or emphysema in AAT deficiency, so aggressive treatment is warranted 1
- If the precise cause cannot be established initially, maintain close follow-up and do not hesitate to repeat the biopsy if the clinical picture changes 3
- Submit biopsy tissue for microbiological analysis and T-cell clonal expansion studies when T-cell lymphoma is suspected 3