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HIV-associated lipodystrophy commonly presents with fat loss in face, buttocks, arms and legs.
There is also fat "accumulation" in various body parts. Patients often present with "buffalo hump"-like fat deposits in their upper backs. Breast size of patients (both male and female) tends to increase. In addition, patients develop abdominal obesity.
Localized lipodystrophy is a skin condition characterized by the loss subcutaneous fat localized to sites of insulin injection.
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.
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. This condition has been described in children who suck ice or popsicles, and therefore is sometimes referred to as "popsicle panniculitis." The term was coined when a patient with a rash of unknown origin on her cheek was taken to a dermatologist.
- "Gouty panniculitis" is a panniculitis caused by deposition of uric acid crystals in gout.
- "Pancreatic panniculitis" (also known as "Enzymatic panniculitis," "Pancreatic fat necrosis," and "Subcutaneous fat necrosis") is a panniculitis most commonly associated with pancreatic carcinoma, and more rarely with anatomic pancreatic abnormalities, pseudocysts, or drug-induced pancreatitis.
- "Factitial panniculitis" is a panniculitis that may be induced by the injection of organic materials, povidone, feces, saliva, vaginal fluid, and oils.
Pain may be the first noticed symptom. People with lipodermatosclerosis have tapering of their legs above the ankles, forming a constricting band resembling an inverted champagne bottle. In addition, there may be brownish-red pigmentation and induration.
Lipoatrophia annularis (also known as "Ferreira–Marques lipoatrophia") is a skin condition affecting primarily women, characterized by the loss of subcutaneous fat in the upper extremity.
It is a form of lipodystrophy.
Centrifugal abdominal lipodystrophy (also known as "Centrifugal lipodystrophy," "Lipodystrophia centrifugalis abdominalis infantalis") is a skin condition characterized by areas of subcutaneous fat loss that slowly enlarge.
HIV-associated lipodystrophy, also known as lipodystrophy in HIV-infected patients (LD-HIV), is a condition characterized by loss of subcutaneous fat associated with infection with HIV.
Septal panniculitis is a condition of the subcutaneous fat affecting the layer of adipose tissue that lies between the dermis and underlying fascia, of which there are two forms: acute erythema nodosum and chronic erythema nodosum.
The clinical presentation is similar to people with congenital lipodystrophy: the only difference is that AGL patients are born with normal fat distribution and symptoms develop in childhood and adolescence years and rarely begins after 30 years of age. Females are more often affected than males, with ratio being 3:1.
The hallmark characteristics are widespread loss of subcutaneous fat, ectopic fat deposition, leptin deficiency, and severe metabolic abnormalities such as insulin resistance. Subcutaneous fat loss in AGL patients are visible in all parts of the body. AGL mostly affects face and the extremities and may look sunken or swollen in the eyes. However, the degree and location of severity may vary by person. Especially, intra-abdominal fat loss is variable. As subcutaneous fat is lost, affected areas show prominent structures of veins and muscle. Those with panniculitis-associated AGL may present erythematous nodules.
Metabolic complications include insulin resistance, high metabolic rate, and uncontrolled lipid levels such as hypertriglyceridemia, low HDL, and high LDL. Patients may develop diabetes mellitus secondary to insulin resistance.
Recent case reports reveled that lymphoma is present in some patients but its prevalence is not known at this time.
Lipodermatosclerosis (also known as "chronic panniculitis with lipomembranous changes", "hypodermitis sclerodermiformis", "sclerosing panniculitis", and "stasis panniculitis") is a skin and connective tissue disease. It is a form of lower extremity panniculitis, an inflammation of the layer of fat under the epidermis.
Acquired generalized lipodystrophy (also known as "Lawrence syndrome," and "Lawrence–Seip syndrome", abbreviation: AGL) is a rare skin condition that appears during childhood or adolescence, characterized by fat loss affecting large areas of the body, particularly the face, arms, and legs. There are 4 types of lipodystrophy based on its onset and areas affected: acquired or inherited (congenital or familial), and generalized or partial. Both acquired or inherited lipodystrophy present as loss of adipose tissues. The near-total loss of subcutaneous adipose tissue is termed generalized lipodystrophy while the selective loss of adipose tissues is denoted as partial lipodystrophy. Thus, as the name suggests, AGL is a near-total deficiency of adipose tissues in the body that is developed later in life. It is an extremely rare disease that only about 100 cases are reported worldwide. There are three main etiologies of AGL suspected: autoimmune, panniculitis-associated, or idiopathic. After its onset, the disease progresses over a few days, weeks, months, or even in years. Clinical presentations of AGL are similar to other lipodystrophies, including metabolic complications and hypoleptinemia. Treatments are also similar and mainly supportive for symptomatic alleviation. Although HIV- or drug-induced lipodystrophy are a type of acquired lipodystrophy, its origin is very specific and distinct hence is usually not discussed with AGL (see HIV-Associated Lipodystrophy).
Weber–Christian disease, also known as relapsing febrile nodular nonsuppurative panniculitis, is a cutaneous condition characterized by recurrent subcutaneous nodules that heal with depression of the overlying skin.
It is type of panniculitis.
It is a rare disease seen in females 30–60 years of age. It is a recurring inflammation of fatty layers of tissue present beneath the skin. Clinical course is characterised by exacerbations and remissions. Lesions are bilaterally symmetrical and are usually seen in the lower legs.
Neutrophilic lobular panniculitis is a cutaneous condition characterized by inflammation of the subcutaneous fat.
Lipoatrophic diabetes is a type of diabetes mellitus presenting with severe lipodystrophy in addition to the traditional signs of diabetes.
Barraquer–Simons syndrome (or acquired partial lipodystrophy, cephalothoracic lipodystrophy, and progressive lipodystrophy)) is a rare form of lipodystrophy,
which usually first affects the head, and then spreads to the thorax.
It is named for Luis Barraquer Roviralta (1855–1928), a Spanish physician, and Arthur Simons (1879–1942), a German physician. Some evidence links it to "LMNB2".
Atrophic connective tissue panniculitis is a rare condition, and often occurs on the upper or lower extremities.
Lipodystrophy can be divided into the following types:
- Congenital lipodystrophies
- Congenital generalized lipodystrophy (Beradinelli-Seip syndrome)
- Familial partial lipodystrophy
- Marfanoid–progeroid–lipodystrophy syndrome
- Acquired lipodystrophy
- Acquired partial lipodystrophy (Barraquer-Simons syndrome)
- Acquired generalized lipodystrophy
- Centrifugal abdominal lipodystrophy (Lipodystrophia centrifugalis abdominalis infantilis)
- Lipoatrophia annularis (Ferreira-Marques lipoatrophia)
- Localized lipodystrophy
- HIV-associated lipodystrophy
Lipodystrophy is a disorder in which the body is unable to produce fat. The medical condition is characterized by abnormal or degenerative conditions of the body's adipose tissue. ("Lipo" is Greek for "fat", and "dystrophy" is Greek for "abnormal or degenerative condition".) A more specific term, "lipoatrophy", is used when describing the loss of fat from one area (usually the face). This condition is also characterized by a lack of circulating leptin which may lead to osteosclerosis.
A review published in 2004, which was based on 35 patients seen by the respective authors over 8 years and also a literature review of 220 cases of acquired partial lipodystrophy (APL), proposed an essential diagnostic criterion. Based on the review and the authors experience, they proposed that APL presents as a gradual onset of bilaterally symmetrical loss of subcutaneous fat from the face, neck, upper extremities, thorax, and abdomen, in the "cephalocaudal" sequence, sparing the lower extremities. The median age of the onset of lipodystrophy was seven years. Several autoimmune diseases, in particular systemic lupus erythematosus and dermatomyositis, were associated with APL. The prevalence rates of diabetes mellitus and impaired glucose tolerance were 6.7% and 8.9%, respectively. Around 83% of APL patients had low complement 3 (C3) levels and the presence of polyclonal immunoglobulin C3 nephritic factor. About 22% of patients developed membranoproliferative glomerulonephritis (MPGN) after a median of about 8 years following the onset of lipodystrophy. Compared with patients without renal disease, those with MPGN had earlier age of onset of lipodystrophy (12.6 ± 10.3 yr vs 7.7 ± 4.4 yr, respectively; p < 0.001) and a higher prevalence of C3 hypocomplementemia (78% vs 95%, respectively; p = 0.02).
The adipose stores of the gluteal regions and lower extremities (including soles) tend to be either preserved or increased, particularly among women. Variable fat loss of the palms, but no loss of intramarrow or retro-orbital fat, has been demonstrated.
Drug-induced lipodystrophy is a cutaneous condition that presents as one or multiple depressed areas (i.e. indentations), usually on the proximal extremities, ranging from under a few centimeters to greater than 20 cm in diameter.
Perforating calcific elastosis (also known as "Localized acquired cutaneous pseudoxanthoma elasticum," "Perforating periumbilical calcific elastosis," and "Periumbilical perforating pseudoxanthoma elasticum") is an acquired, localized cutaneous disorder, most frequently found in obese, multiparous, middle-aged women, characterized by lax, well-circumscribed, reticulated or cobble-stoned plaques occurring in the periumbilical region with keratotic surface papules.
The first signs of erythema nodosum are often flu-like symptoms such as a fever, cough, malaise, and aching joints. Some people also experience stiffness or swelling in the joints and weight loss.
Erythema nodosum is characterised by nodules (rounded lumps) below the skin surface, usually on the shins. These subcutaneous nodules can appear anywhere on the body, but the most common sites are the shins, arms, thighs, and torso. Each nodule typically disappears after around two weeks, though new one may continue to form for up to six or eight weeks. A new nodule usually appears red and is hot and firm to touch. The redness starts to fade and it gradually becomes softer and smaller until it disappears. Each nodule usually heals completely without scarring over the course of about two weeks. Joint pain and inflammation sometimes continues for several weeks or months after the nodules appear.
Less common variants of erythema nodosum include:
- Ulcerating forms, seen in Crohn's disease
- Erythema contusiforme, when a subcutaneous hemorrhage (bleeding under the skin) occurs with a erythema nodosum lesion, causing the lesion to look like a contusion (bruise)
- Erythema nodosum migrans (also known as subacute nodular migratory panniculitis), a rare form of chronic erythema nodosum characterized by asymmetrical nodules that are mildly tender and migrate over time.
Dunnigan-type familial partial lipodystrophy, also known as FPLD Type II and abbreviated as (FPLD2), is a rare monogenic form of insulin resistance characterized by loss of subcutaneous fat from the extremities, trunk, and gluteal region. FPLD recapitulates the main metabolic attributes of the insulin resistance syndrome, including central obesity, hyperinsulinemia, glucose intolerance and diabetes usually type 2, dyslipidemia, hypertension, and early endpoints of atherosclerosis. It can also result in hepatic steatosis. FPLD results from mutations in LMNA gene, which is the gene that encodes nuclear lamins A and C.