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Panniculitis

Panniculitis is a group of diseases whose hallmark is inflammation of subcutaneous adipose tissue. Symptoms include tender skin nodules, and systemic signs such as weight loss and fatigue.

Signs and symptoms
Panniculitis can also be classified based on the presence or absence of systemic symptoms. Panniculitis without systemic disease can be a result of trauma or cold. Panniculitis with systemic disease can be caused by: • 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 deficiencyCrohn'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. ==Diagnosis==
Diagnosis
Classification It can occur in any fatty tissue (cutaneous or visceral) and is often diagnosed based on a deep skin biopsy, and can be further classified by histological characteristics based on the location of the inflammatory cells (within fatty lobules or in the septa which separate them) and on the presence or absence of vasculitis. There are thus four main histological subtypes: • lobular panniculitis without vasculitis (acute panniculitis, previously termed Weber–Christian disease, systemic nodular panniculitis) • lobular panniculitis with vasculitis • septal panniculitis without vasculitis • septal panniculitis with vasculitis Lobular With vasculitis Erythema induratum, or "Bazin disease", is a panniculitis on the back of the calves. It was formerly thought to be a reaction to the tuberculum bacillus. It is now considered a panniculitis that is not associated with a single defined pathogen. 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 manifestations of tuberculosis, and indeed they respond well to anti-tuberculous treatment. Without vasculitis Non-vasculitis forms of panniculitis that may occur include: • Cytophagic histiocytic panniculitis was first described in 1980 by Winkelmann as a chronic histiocytic disease of the subcutaneous adipose tissue, which is characterized clinically by tender erythematous nodules, recurrent high fever, malaise, jaundice, organomegaly, serosal effusions, pancytopenia, hepatic dysfunction and coagulation abnormalities. CHP may occur either isolated or as part of cutaneous manifestations of hemophagocytic syndrome (HPS). 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. • Cold panniculitis is a panniculitis occurring after exposure to cold, most often seen in infants and young children. • Gouty panniculitis is a panniculitis caused by deposition of uric acid crystals in gout. Other forms include: • Subcutaneous fat necrosis of the newborn, a form of panniculitis occurring in newborns that is usually self-resolving, that may be a result of hypoxic injury to relatively high levels of brown fat. 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 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. A1AT-deficiency-associated Alpha-1 antitrypsin deficiency panniculitis is a panniculitis associated with a deficiency of the α1-antitrypsin enzyme inhibitor. ==See also==
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