What are the treatment options for managing panniculitis?

Management of Panniculitis

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 systemic corticosteroids are the first-line treatment for most other forms of panniculitis. 1

Diagnosis and Classification

Proper management begins with accurate diagnosis and classification of the panniculitis type:

• Deep skin biopsy is essential for histopathological examination to differentiate between:

  • Septal panniculitis (inflammation primarily in the septa between fat lobules)
  • Lobular panniculitis (inflammation primarily within fat lobules)
  • Presence or absence of vasculitis 2

• Common types of panniculitis:

  1. Erythema nodosum (EN) - most common septal panniculitis
  2. Alpha-1 antitrypsin deficiency panniculitis - distinctive form leading to spontaneous ulcerations
  3. Pancreatic panniculitis - associated with pancreatic disorders
  4. Inflammatory bowel disease (IBD)-associated panniculitis
  5. Vasculitis-associated panniculitis

Treatment Approaches by Type

1. Alpha-1 Antitrypsin Deficiency Panniculitis

• First-line treatment: Augmentation therapy with purified human AAT or fresh frozen plasma 1
• Alternative options:
  • Dapsone (alone for less severe cases or combined with augmentation therapy)
  • Liver transplantation (can lead to permanent cure by restoring plasma AAT levels) 1
• Ineffective treatments: Corticosteroids, antibiotics, and cytostatic drugs 1

2. Erythema Nodosum (EN)

• Treatment based on underlying IBD activity:
  • First-line: Systemic corticosteroids for severe cases 1
  • For resistant/relapsing cases: Immunomodulation with:
    • Azathioprine
    • Infliximab
    • Adalimumab 1

3. Pyoderma Gangrenosum (PG)

• First-line treatment: Systemic corticosteroids 1
• For refractory cases:
  • Infliximab (especially effective with 90% response rate in cases <12 weeks duration) 1
  • Ciclosporin (oral or intravenous)
  • Tacrolimus (oral or intravenous) 1
• For peristomal PG: Consider stoma closure if possible 1
• Topical option: Tacrolimus (with specialist advice) 1

4. Pancreatic Panniculitis

• Primary approach: Treatment of the underlying pancreatic disease 3, 4
• Symptomatic treatment:
  • Octreotide or steroids may provide symptomatic relief
  • However, treating the underlying condition is the only effective management 4

5. Malignancy-Associated Panniculitis

• For lymphoma-associated panniculitis:
  • Etoposide, cyclophosphamide, or methotrexate have shown efficacy 1
  • Cyclosporin A may be effective for clonal cytophagic histiocytic panniculitis 1

6. Immune Checkpoint Inhibitor-Related Panniculitis

• Management based on severity:
  • Early dermatology consultation
  • Topical or systemic corticosteroids
  • Temporary or permanent discontinuation of immune checkpoint therapy for severe cases 1

Special Considerations

• Infectious panniculitis:

  • Early dermatology consultation for patients with immune defects
  • Consider biopsy and surgical debridement
  • Empiric antimicrobials in life-threatening situations 1
  • For Nocardia-associated panniculitis: SMX-TMP is treatment of choice 1

• Monitoring and follow-up:

  • Follow patients closely when the precise cause cannot be established initially
  • Consider repeat skin biopsy if diagnosis remains unclear 2

Common Pitfalls to Avoid

1. Misdiagnosis: Panniculitis can mimic other conditions; ensure proper deep biopsy for accurate diagnosis
2. Delayed treatment: Panniculitis can be the initial presentation of serious underlying conditions (e.g., pancreatic disease, AAT deficiency)
3. Ineffective therapy: Using corticosteroids for AAT deficiency-associated panniculitis is ineffective
4. Overlooking rare causes: Always consider pancreatic disorders, malignancies, and AAT deficiency in the differential diagnosis 3
5. Inadequate biopsy: Ensure deep excisional specimens with adequate tissue for proper histopathological evaluation 1

By targeting the underlying cause while providing appropriate symptomatic relief, most forms of panniculitis can be effectively managed, though some may require long-term therapy or follow-up.