Panniculitis With Vasculitis: A Brief Review

What Is Panniculitis With Vasculitis?

Panniculitis with vasculitis is a medical disorder characterized by inflammation within subcutaneous fat tissue called panniculitis and blood vessel inflammation called vasculitis. The panniculitis and vasculitis components mostly co-exist and can be associated with underlying medical conditions. The condition affects several tissues and organs within the body.

Depending on which fat layer is affected, panniculitis can be categorized as septal, lobular, or mixed. Vasculitis develops when blood vessels become inflamed, disrupts the blood flow, and results in damage to affected organs and tissues. A combination of panniculitis and vasculitis indicates inflammatory processes occurring in fat tissues and blood vessels.

What Are the Different Types of Panniculitis With Vasculitis?

1. Septal Panniculitis With Vasculitis: Septal panniculitis is the inflammation of fibrous septa, which divides subcutaneous fat into lobules. Septal panniculitis with vasculitis affects fibrous septa, subcutaneous fat, and small arteries. Examples include:

• Leukocytoclastic Vasculitis: It is a group of disorders that causes inflammation in blood vessels and the dermis of the skin. The capillaries, lymphatics, venules, and arterioles can be affected.

• Superficial Thrombophlebitis: It is a condition where inflammation develops in the superficial vein due to the formation of blood clots.

• Cutaneous Polyarteritis Nodosa: It is a systemic vasculitis disorder affecting several medium-sized arteries. Panniculitis presents itself as a cutaneous manifestation of the disorder. Painful subcutaneous nodules appear throughout the body.

• Behcet Disease: It is a form of vasculitis primarily affecting small arteries.

2. Lobular Panniculitis With Vasculitis: It is a condition where inflammation develops in lobules of subcutaneous fat and blood vessels. Examples include:

• Erythema Nodosum Leprosum (Leprosy): It is a common disorder developing panniculitis with vasculitis. The characteristics of the condition are painful, erythematous nodules on the shin. Erythema nodosum is caused by inflammatory bowel disease, sarcoidosis, streptococcal infections, or medications.

• Erythema Induratum: It is a cutaneous tuberculosis developing due to Mycobacterium tuberculosis infection.

• Infective Panniculitis: The condition develops when infective agents invade immunocompromised individuals and cause inflammation in subcutaneous fat.

• Nodular Vasculitis: Nodular vasculitis is a chronic type of panniculitis with vasculitis. The condition presents as nodules in the lower extremities. Nodular vasculitis develops in systemic conditions like tuberculosis, rheumatoid arthritis, and specific medications.

• Neutrophilic lobular panniculitis associated with rheumatoid arthritis.

• Crohn's disease.

• Nodular panniculitis.

3. Mixed Panniculitis With Vasculitis: It is a condition where inflammation occurs in both fibrous septa and lobules of subcutaneous fat and blood vessels.

4. Others:

• Acute Febrile Neutrophilic Dermatosis: It is also called sweet syndrome and affects the subcutaneous fat. The condition presents as neutrophilic infiltrate, fever, painful nodule, or plaque.

• Lupus Panniculitis: It is a subtype of cutaneous lupus erythematosus. The condition causes inflammation of subcutaneous fat and vasculitis.

• Traumatic Vasculitis: Trauma and injury can cause panniculitis with vasculitis.

What Are the Causes of Developing Panniculitis With Vasculitis?

Several factors contribute to the development of panniculitis with vasculitis. The condition can appear as a primary condition or may be associated with a systemic disease. Some possible causes include:

1. Connective Tissue Disorder: Rheumatoid arthritis, systemic lupus eythematosus (SLE), and dermatomyositis are often associated with panniculitis and vasculitis.

2. Infections: Viral, bacterial, and fungal infections can cause panniculitis with vasculitis to develop.

3. Autoimmune Disorder: Behcet's disease and polyarteritis nodosa cause both panniculitis and vasculitis to develop.

4. Medications: Antibiotics and immune-modulating medications could trigger panniculitis with vasculitis as a side effect.

5. Idiopathic Nodular Vasculitis: The exact cause for developing the condition remains unknown.

6. Pancreatic Enzyme Abnormalities: Disorders of the pancreas, such as pancreatic panniculitis, cause lobular panniculitis with vasculitis.

What Are the Symptoms of Panniculitis With Vasculitis?

The symptoms could vary, but the common symptoms are the development of painful red or purple plaques or nodules under the skin, accompanied by systemic symptoms like fever, fatigue, and malaise. Severe cases of panniculitis with vasculitis cause ulcers to develop on the skin.

The affected skin regions are painful to touch. The developing subcutaneous nodules are palpable on the skin. Some affected individuals develop joint pain or skin discoloration. Rarely do individuals develop Raynaud’s phenomenon, where the skin turns white or blue as a response to cold. At times, internal organs get affected, manifesting symptoms specific to the organ.

How Is Panniculitis With Vasculitis Diagnosed?

A combination of clinical symptoms, skin biopsy, imaging studies, and laboratory tests helps determine the underlying cause for developing the condition. Doctors record medical history and conduct physical examinations to analyze the disease. Blood tests such as erythrocyte sedimentation rate and C-reactive protein help identify inflammatory markers. Other autoimmune markers like rheumatoid factor and antinuclear antibodies are tested to analyze the underlying cause for developing the condition. Imaging studies like ultrasound, MRI (magnetic resonance imaging), and CT (computed tomography) scans help visualize affected skin and the extent of inflammation. Diagnosing the specific type of panniculitis requires histopathological examination to determine the disease. Biopsy of the skin or subcutaneous tissue is vital for diagnosing the condition. Biopsy reveals inflammation within blood vessels and subcutaneous fat tissue when examined under a microscope.

How Is Panniculitis With Vasculitis Treated?

• Disease management depends on the underlying cause responsible for the condition. The treatment aims to manage primary disease, immunosuppressive medication, and supportive care.
• Corticosteroid medications such as Prednisone help reduce inflammation and suppress the immune response. If steroids are ineffective or not well tolerated, then immunosuppressive medications like Methotrexate are given.
• Panniculitis with vasculitis associated with autoimmune disorders requires treatment with biological therapies like Rituximab. Pain associated with lesions in the disease is eased with nonsteroidal anti-Inflammatory drugs (NSAIDs).
• If rheumatoid arthritis is the cause for developing the condition, then disease-modifying antirheumatic drugs are prescribed (DMARDs) to control inflammation and prevent disease progression. Infectious causes of panniculitis with vasculitis are treated with antibiotics therapy.
• Open lesions or ulcerations that develop in severe panniculitis must be treated adequately. After cleaning the wound, topical treatment should be used, and a dressing should be placed on it.
• Lifestyle modifications are essential for individuals suffering from panniculitis with vasculitis. Smoking cessation is crucial as it can exacerbate inflammation. Physical therapy helps improve symptoms and keep joints healthy.

Conclusion

Panniculitis with vasculitis is a medical condition causing inflammation within subcutaneous tissue and blood vessels. The symptoms include the appearance of painful nodules on the skin. Skin biopsy is crucial for diagnosing the disorder. Treatment is aimed at curing the underlying disease and immunosuppressive therapy.