Introduction - panniculitis Introduction Demographics Causes Clinical features Complications Diagnosis Treatment Outlook
Panniculitis is a condition where the fat under the skin becomes inflamed.
Nodular panniculitis is characterised by one or multiple, recurrent, subcutaneous nodules. These commonly arise on the legs but can arise elsewhere on the body. Nodular panniculitis is associated with acute systemic symptoms such as fever, general malaise and abdominal pain. The term 'nodular panniculitis' is used when no specific cause for the panniculitis has been found (such as erythema nodosum or alpha-1-antitrypsin deficiency).
Nodular panniculitis is also referred to as relapsing, febrile, non-suppurative panniculitis. In the past, it was also called Weber Christian disease.
Nodular panniculitis is rare. It occurs in males and females of all ages. It most commonly affects young adult women, and it rarely affects young children.
Nodular panniculitis is associated with autoimmune diseases such as Sjögren syndrome, inflammatory bowel disease, systemic lupus erythematosus, and diabetes mellitus.
The cause of nodular panniculitis is unknown. One hypothesis is that fat may trigger an autoimmune or autoinflammatory granulomatous reaction.
The mechanism of nodular panniculitis is that white blood cells infiltrate and damage the subcutaneous fat, causing necrosis and fibrosis.
The first signs of nodular panniculitis are:
Each nodule regresses after a few weeks. In some cases, the nodules can ulcerate and leak an oily, yellow discharge.
Nodular panniculitis can involve visceral organs and cause other symptoms and complications such as:
Skin biopsy shows a lobular pattern of inflammation with or without vasculitis. Nodular panniculitis is diagnosed when other forms of lobular panniculitis associated with fever have been excluded.
There is no single effective therapy. Nodular panniculitis without the involvement of other organs should be treated symptomatically.
Other treatments reported to be of benefit include mycophenolate mofetil, thalidomide, clofazimine, antimalarials such as hydroxychloroquine, antibiotics, dapsone, and immunosuppressive drugs such as azathioprine and ciclosporin.
Nodular panniculitis tends to settle down after a few weeks or months. There may be a single episode, or the disease may relapse repeatedly over time.
Involvement of other organs is associated with a high risk of mortality.
