Panniculitis

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Panniculitis
Erythema induratum, a form of lobular panniculitis
Specialty	Rheumatology

Panniculitis is a group of diseases whose hallmark is

Restated, an inflammatory disorder primarily localized in the subcutaneous fat is termed a "panniculitis", a group of disorders that may be challenging both for the clinician and the dermatopathologist.^[2]: 487 The general term for inflammation of any adipose tissue is steatitis.

Signs and symptoms

Panniculitis can also be classified based on the presence or absence of systemic

• connective tissue disorders such as lupus erythematosus or scleroderma;
• lymphoproliferative disease such as lymphoma or histiocytosis
  ;
• pancreatitis or pancreatic cancer;
• sarcoidosis with cutaneous involvement (seen in up to 20 percent);
• Alpha 1-antitrypsin deficiency
• Crohn's disease

This is not a complete list of possible causes.

Associated conditions

Lipoatrophy or lipodystrophy (the loss of subcutaneous adipose tissue) can occur in any of these conditions.^{[citation needed]}

Diagnosis

Classification

It can occur in any fatty tissue (

There are thus four main histological subtypes:[4]

1. lobular panniculitis without vasculitis (acute panniculitis, previously termed Weber–Christian disease,^[5] systemic nodular panniculitis)
2. lobular panniculitis with vasculitis
3. septal panniculitis without vasculitis
4. septal panniculitis with vasculitis

Lobular

With vasculitis

Nodular vasculitis is a skin condition characterized by small, tender, reddened nodules on the legs, mostly on the calves and shins. Microscopically there are epithelioid granulomas and vasculitis in the subcutaneous tissue, making it a form of panniculitis. Most of these cases are now thought to be manifestation of tuberculosis and indeed they respond well to anti-tuberculous treatment.^{[citation needed]}

Without vasculitis

Non-vasculitis forms of panniculitis that may occur include:

• Cytophagic histiocytic panniculitis was first described in 1980 by Winkelmann as a chronic
  hemophagocytic syndrome (HPS).^[9]
  CHP is a rare and often fatal form of panniculitis with multisystem involvement. But it can also present in a benign form involving only the subcutaneous tissue, thus having a broad clinical spectrum.
• Traumatic panniculitis is a panniculitis that occurs following trauma to the skin.[2]^{: 492 [10]}
• Cold panniculitis is a panniculitis occurring after exposure to cold, most often seen in infants and young children.
  dermatologist.^[11]
• Gouty panniculitis is a panniculitis caused by deposition of uric acid crystals in gout.^[2]: 494
• Pancreatic panniculitis (also known as enzymatic panniculitis, Pancreatic fat necrosis,
  pancreatic carcinoma, and more rarely with anatomic pancreatic abnormalities, pseudocysts, or drug-induced pancreatitis.^[2]
  ^: 493
• Factitial panniculitis is a panniculitis that may be induced by the injection of organic materials,
  vaginal fluid, and oils.^[2]
  ^: 492

Lipodermatosclerosis is a form of panniculitis associated with chronic venous insufficiency that presents with brown indurations on the front of the shins. It may be associated with pain and other signs of chronic venous insufficiency. The exact cause is unknown.^[12]

Other forms include:

• brown fat.^[2]
  ^: 492
• premature births.^[2]
  ^: 492
• Weber–Christian disease, a symmetrical form of the disease of unknown origin occurring in middle-aged women.^[2]: 492
• Lupus erythematosus panniculitis, panniculitis associated with lupus erythematosus.^[13]
• Forms associated with use of high doses of systemic corticosteroids during rapid corticosteroid withdrawal, and from the injection of silicone or mineral oils.^{[2]: 492 [14]}

Septal

Erythema nodosum

Erythema nodosum is a form of panniculitis characterised by tender red nodules, 1–10 cm, associated with systemic symptoms including fever, malaise, and joint pain. Nodules may become bluish-purple, yellowing, and green, and subside over a period of 2–6 weeks without ulcerating or scarring. Erythema nodosum is associated with infections, including Hepatitis C, EBV and tuberculosis, Crohn's disease and sarcoidosis, pregnancy, medications including sulfonamides, and some cancers, including Non-Hodgkin lymphoma and pancreatic cancer.^[15]

A1AT-deficiency-associated

Alpha-1 antitrypsin deficiency panniculitis^[13] is a panniculitis associated with a deficiency of the α₁-antitrypsin enzyme inhibitor.^[2]: 494

Treatment

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See also

• Panniculus carnosus
• List of cutaneous conditions

References