What is the treatment for panniculitis?

Treatment of Panniculitis

The treatment of panniculitis should be guided by the specific type of panniculitis, with alpha-1 antitrypsin (AAT) deficiency testing being essential in all cases of biopsy-proven severe panniculitis, as augmentation therapy with purified human AAT or fresh frozen plasma is the most effective treatment for AAT deficiency-associated panniculitis. 1

Diagnosis and Classification

Before initiating treatment, proper diagnosis is crucial:

1. Deep excisional biopsy: Essential for histopathological evaluation to determine the type of panniculitis (septal vs. lobular, with or without vasculitis) 1
2. Laboratory testing:
   • Alpha-1 antitrypsin (AAT) levels for all cases of severe panniculitis 1
   • Additional tests based on clinical suspicion (inflammatory markers, specific infections)

Treatment Algorithm

1. AAT Deficiency-Associated Panniculitis

• First-line: Augmentation therapy with purified human AAT or fresh frozen plasma 1
• Alternative: Dapsone alone or combined with augmentation therapy for less severe cases 1
• Note: Corticosteroids, antibiotics, and cytostatic drugs are ineffective in this form 1

2. Erythema Nodosum (Most Common Septal Panniculitis)

• First-line: Treatment of the underlying IBD or other triggering condition 2
• Second-line: Systemic corticosteroids for severe cases 2
• Refractory cases: Immunomodulators (azathioprine) or anti-TNF agents (infliximab, adalimumab) 2
• Note: Even without specific therapy, EN typically resolves spontaneously 3

3. Pyoderma Gangrenosum (PG)

• Goal: Rapid healing to prevent debilitating outcomes 2
• First-line: Systemic corticosteroids 2
• Refractory cases:
  • Infliximab (particularly effective with >90% response in PG <12 weeks duration) 2
  • Cyclosporine or tacrolimus (oral/IV) 2
• Topical option: Tacrolimus for localized cases 2

4. Other Types of Panniculitis

• Initial treatment: Prednisone 1 mg/kg/day (up to 80 mg/day) for 1 month before tapering 1
• Severe cases: IV pulse methylprednisolone 500-1000 mg/day for 3-5 days before transitioning to oral prednisone 1
• Tapering schedule: Aim for low-dose (≤10 mg/day) or complete withdrawal if possible 1

5. Special Cases

Clonal Cytophagic Histiocytic Panniculitis

• Cyclosporin A has shown efficacy 2

Subcutaneous Panniculitis-like T-cell Lymphoma with HLH Features

• Cyclosporin A may be effective 2

Malignancy-Associated Panniculitis

• Treatment depends on the underlying malignancy 2
• Etoposide has shown better survival outcomes in secondary HLH compared to treatment directed only at the underlying pathology 2

Monitoring and Follow-up

• Assess for new lesions and healing of existing lesions every 1-2 weeks during initial treatment 1
• Monitor for steroid-related adverse effects if using corticosteroids:
  • Blood pressure
  • Blood glucose
  • Weight changes
  • Mood alterations
  • Signs of infection 1

Common Pitfalls to Avoid

1. Failure to test for AAT deficiency in severe panniculitis cases, leading to inappropriate treatment 1
2. Inappropriate steroid use in AAT deficiency-associated panniculitis (ineffective and potentially harmful) 1
3. Inadequate biopsy specimens - large-scalpel incisional biopsies are required for proper diagnosis 4
4. Delayed treatment of PG - rapid healing should be the goal as it can become debilitating 2
5. Overlooking drug-induced panniculitis - common causative drugs include oral contraceptives, NSAIDs, antibiotics, and leukotriene-modifying agents 5

By following this structured approach based on the specific type of panniculitis, clinicians can provide effective treatment while minimizing complications and improving outcomes.