Treatment Approach for Panniculitis
The treatment of panniculitis should be directed at the underlying cause, with augmentation therapy using purified human alpha-1 antitrypsin (AAT) or fresh frozen plasma being the most effective approach for AAT deficiency-associated panniculitis, while corticosteroids combined with immunosuppressive agents are recommended for other forms of panniculitis. 1
Diagnosis and Classification
Before initiating treatment, proper diagnosis is essential:
- Obtain a deep excisional biopsy with adequate tissue for histopathological evaluation, as superficial biopsies may miss the pathology in medium-sized vessels of the dermis 1
- Classify the panniculitis as predominantly septal or lobular, and determine whether vasculitis is present 2, 3
- Test for alpha-1 antitrypsin deficiency in all cases of biopsy-proven severe panniculitis, particularly in factitious and necrotizing cases 1
- Consider imaging studies to evaluate extent of disease, especially in cases with subcutaneous presentation 1
Treatment Based on 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 plasma and local tissue levels of AAT 1, 4
- Dapsone may be beneficial, either alone in less severe cases or combined with augmentation therapy 1
- Family screening and antismoking counseling are essential components of management 1
- Corticosteroids, antibiotics, and cytostatic drugs appear ineffective for this form of panniculitis 1
- In severe cases, liver transplantation has led to permanent cure by restoring plasma AAT levels 1
Vasculitis-Associated Panniculitis
- For polyarteritis nodosa with cutaneous involvement, treatment with cyclophosphamide and high-dose glucocorticoids is recommended over high-dose glucocorticoids alone 1
- For severe disease, initiate treatment with intravenous pulse glucocorticoids over high-dose oral glucocorticoids 1
- In patients unable to tolerate cyclophosphamide, other non-glucocorticoid immunosuppressive agents should be considered 1
Other Forms of Panniculitis
- Systemic corticosteroids combined with methotrexate and thalidomide have shown efficacy in breast panniculitis 5
- For panniculitis associated with malignancy, treatment should address both the HLH (hemophagocytic lymphohistiocytosis) and the underlying neoplasm 1
- Cyclosporin A has shown efficacy in clonal cytophagic histiocytic panniculitis and subcutaneous panniculitis-like T-cell lymphoma with features of HLH 1
Monitoring and Follow-up
- For patients with a history of severe panniculitis with abdominal involvement who become clinically asymptomatic, follow-up abdominal vascular imaging is recommended 1
- For patients with peripheral motor neuropathy secondary to panniculitis, serial neurologic examinations are preferred over repeated electromyography/nerve conduction studies 1
- Regular follow-up is important as the precise cause of panniculitis may not be established initially, and repeat biopsies may be necessary 2
Common Pitfalls and Caveats
- Inadequate biopsy depth is a common pitfall in diagnosis; ensure deep excisional specimens are obtained 1
- Panniculitis may be misdiagnosed as Weber-Christian disease, which is a diagnosis of exclusion 1, 6
- The changing evolutionary nature of panniculitis lesions means that biopsy specimens are often taken from late-stage lesions, resulting in nonspecific findings 7
- Treatment may be challenging when the precise cause cannot be established initially; close follow-up and repeated biopsies may be necessary 2
- Panniculitis can be lethal, especially when associated with other complications such as cirrhosis or emphysema in AAT deficiency 1