What is the treatment for panniculitis?

Treatment of Panniculitis

Treatment of panniculitis must be tailored to the specific underlying etiology, with alpha-1 antitrypsin deficiency-associated panniculitis requiring augmentation therapy, vasculitis-associated forms requiring cyclophosphamide and high-dose glucocorticoids, and malignancy-associated panniculitis requiring treatment of both the hemophagocytic lymphohistiocytosis and the underlying neoplasm. 1

Diagnostic Approach Before Treatment

• Obtain a deep excisional biopsy with adequate tissue for histopathological evaluation, as superficial biopsies miss pathology in medium-sized vessels of the dermis 1
• Test for alpha-1 antitrypsin deficiency in all cases of biopsy-proven severe panniculitis, particularly in factitious and necrotizing cases 1
• Submit tissue for microbiological analysis and T-cell clonal expansion testing if T-cell lymphoma is suspected 2
• Classify panniculitis as predominantly septal versus lobular, and determine presence or absence of vasculitis 2

Etiology-Specific Treatment Algorithms

Alpha-1 Antitrypsin Deficiency-Associated Panniculitis

Augmentation therapy with purified human alpha-1 antitrypsin or fresh frozen plasma is the most effective treatment, as it restores plasma and local tissue levels of AAT. 1

• Initiate augmentation therapy as first-line treatment for severe cases 1
• Add dapsone either alone in less severe cases or combined with augmentation therapy 1
• Provide antismoking counseling and family screening as essential management components 1
• Consider liver transplantation in severe cases, which has led to permanent cure by restoring plasma AAT levels 1

Vasculitis-Associated Panniculitis (Polyarteritis Nodosa with Cutaneous Involvement)

• Initiate treatment with intravenous pulse glucocorticoids combined with cyclophosphamide for severe disease 1
• Use intravenous pulse glucocorticoids over high-dose oral glucocorticoids for severe presentations 1
• Substitute other non-glucocorticoid immunosuppressive agents in patients unable to tolerate cyclophosphamide 1
• Perform follow-up abdominal vascular imaging for patients with history of severe panniculitis with abdominal involvement who become clinically asymptomatic 1

Malignancy-Associated Panniculitis

Treatment must address both the hemophagocytic lymphohistiocytosis and the underlying neoplasm. 1

• Use cyclosporin A for clonal cytophagic histiocytic panniculitis and subcutaneous panniculitis-like T-cell lymphoma with features of hemophagocytic lymphohistiocytosis 3, 1
• Consider etoposide-containing regimens, as etoposide has the greatest clinical experience and treats both HLH and underlying neoplasm 3
• Follow HLH-directed therapy with neoplasm-directed protocol when HLH resolves 3
• Consider anti-B-cell therapy (rituximab) for highly replicative EBV infection associated with HLH 3

Erythema Nodosum (Most Common Form)

• Provide symptomatic support alone for the majority of patients, as erythema nodosum typically resolves without specific treatment 4
• Identify and treat underlying triggers including infections, drugs, sarcoidosis, inflammatory bowel disease, pregnancy, or malignancy 4
• Recognize that up to 55% of cases are idiopathic and self-limited 4

Other Forms Requiring Specific Approaches

• Use systemic corticosteroids combined with methotrexate and thalidomide for breast panniculitis with liquefactive fat necrosis 5
• Employ anti-inflammatory and immunosuppressive agents after eliminating the underlying cause 6
• Reserve surgery for cases of recurrent bowel obstruction in mesenteric panniculitis 7
• Use prednisone and tamoxifen as first-line medical treatment for symptomatic mesenteric panniculitis 7

Monitoring and Follow-Up

• Perform serial neurologic examinations rather than repeated electromyography/nerve conduction studies for patients with peripheral motor neuropathy secondary to panniculitis 1
• Repeat skin biopsy if the precise cause cannot be established initially or if clinical course changes 2

Critical Pitfalls to Avoid

• Inadequate biopsy depth is the most common diagnostic pitfall—always obtain deep excisional specimens 1
• Panniculitis can be lethal, especially when associated with cirrhosis or emphysema in AAT deficiency 1
• Do not assume all panniculitis is benign—always exclude malignancy-associated forms and T-cell lymphoma 2
• In malignancy-associated HLH, failing to treat both the HLH and underlying neoplasm leads to poor outcomes 3